Human immune status, assessment methods
Main questions
1.Immune status and its disorders.
2.Immunopathological syndromes.
3.Immunological tests of levels 1 and 2.
4.Rules for evaluating immunograms.
5. Methods for assessing lymphocytes.
1

Immune status

Immune status is a quantitative and
qualitative characteristics of the condition
functional activity of organs
immune system and some
nonspecific mechanisms
antimicrobial protection.
2

Immune status is determined by effectiveness
and consistency of operation of all systems and
links of immunity - macrophages,
complement, cytokines, T- and B-lymphocytes,
major histocompatibility system.
Branch of medicine that studies pathology
person in terms of dysfunction
immune system, called clinical
immunology.
3

The study of immune status includes:

1) determination of blood group and Rh factor;
2) general analysis blood with a detailed leukogram or
formula;
3) determination of the amount of immunoglobulins;
4) study of lymphocytes;
5) study of the phagocytic activity of neutrophils.
To make a diagnosis of immunopathological
conditions are carried out: collecting an immunological history,
setting up clinical laboratory, instrumental and
immunological tests.
4

History taking
During the survey, the probable
immunopathological syndrome, the main
are:
- infectious syndrome;
- allergic and autoimmune syndromes;
- primary immunodeficiency;
- secondary immunodeficiency;
- immunoproliferative syndrome.
5

- taking into account possible individual
characteristics (age, associated
diseases) and fluctuations in indicators
(physiological and pathological - reception
food, exercise, time of day,
the effect of stressors, etc.);
- taking into account regional standards;
6

General rules when assessing immunograms:
- a comprehensive analysis, rather than an assessment of one
indicator;
- analysis in combination with clinical and
anamnestic data;
- assessment of sharp shifts in indicators (not
less than 20% of the norm);
- analysis in dynamics;
- analysis not only (and not so much)
absolute data, but ratios
indicators (especially Th/Ts index);
7

Petrov R.V. et al. created a two-step approach to
assessment of immune status, according to which
immunological tests divided into tests
first and second levels.
At the first stage, using simple methods
reveal “gross” defects in phagocytosis, cellular
and humoral immunity.
The first level tests include:
- determination of the number of lymphocytes in the blood (abs., rel.);
- determination of the number of T- and B-lymphocytes;
- determination of the level of Ig classes IgG, IgM, IgA;
- determination of phagocytic activity of leukocytes;
- determination of complement titer.
Taking into account the analysis of the results, it is determined
further research tactics.
8

Leukocytes

The norm is 3.5–8.8 4 109/l. Increased number of leukocytes –
this is leukocytosis, a decrease is leukopenia. Leukocytosis
divided into physiological and pathological.
physiological leukocytosis may be food intake,
physical work, taking hot and cold baths,
pregnancy, childbirth, premenstrual period.
Pathological leukocytosis occurs with infectious
diseases (pneumonia, meningitis, general sepsis and
etc.), infectious diseases with cell damage
immune system. But there are also exceptions. For example,
Some infectious diseases occur with
leukopenia ( typhoid fever, brucellosis, malaria,
rubella, measles, influenza, viral hepatitis in the acute phase).
9

Lymphocytes

Norm: absolute content – ​​1.2–3.0 109/l, but more often
in a clinical blood test the percentage is indicated
lymphocyte content.
This figure is 19–37%.
Lymphocytosis is found in chronic
lymphocytic leukemia, chronic radiation sickness,
bronchial asthma, thyrotoxicosis, some
infectious diseases (whooping cough, tuberculosis),
when removing the spleen.
Developmental anomalies lead to lymphopenia
lymphoid system, viral infections,
ionizing radiation, autoimmune diseases
(systemic lupus erythematosus), endocrine diseases
(Cushing's disease, taking hormonal drugs),
AIDS.
10

T lymphocytes

Norm: relative content 50–
90%, absolute – 0.8–2.5 109/l.
The number of T lymphocytes increases with
allergic diseases, during
recovery for tuberculosis. Decline
content of T-lymphocytes occurs when
chronic infections, immunodeficiencies,
tumors, stress, trauma, burns,
some forms of allergies, heart attack.
11

T helper cells

Norm: relative content – ​​30–
50%, absolute – 0.6–1.6 109/l.
The content of T-helper cells increases with
infections, allergic diseases,
autoimmune diseases
(rheumatoid arthritis, etc.). Decline
content of T-helper cells occurs when
immunodeficiency states, AIDS,
cytomegalovirus infection.
12

B lymphocytes

Norm: relative content – ​​10–
30%, absolute – 0.1–0.9 in 109/l.
Increased content occurs when
infections, autoimmune diseases,
allergies, lymphocytic leukemia.
Decrease in the number of B lymphocytes
found in immunodeficiencies,
tumors.
13

Phagocytes (neutrophils)

Their activity is assessed using methods that
determine the part of cells capable of forming within themselves
phagosome.
To assess the digestive capacity of neutrophils
use the NBT test (NBT is a nitro blue dye
tetrazolium).
The norm of the NST test is 10–30%. Phagocytic activity
leukocyte counts increase during acute bacterial infections,
decreases in congenital immunodeficiencies, chronic
infections, autoimmune diseases, allergies, viral
infections, AIDS.
The activity of phagocytes is assessed by the so-called
phagocytic number (normally the cell absorbs 5-10
microbial particles), number of active phagocytes, index
completeness of phagocytosis (must be greater than 1.0).
14

Methods for studying lymphocytes

Study of surface CD antigens
It is based on:
methods of rosette formation;
flow cytometry method;
immunofluorescence methods;
enzyme immunoassay.
Functional tests include assessment methods
proliferative activity of lymphocytes on T- and
B-mitogens (RBTL- blast reaction
transformation of lymphocytes), synthesis
cytokine mononuclear cells.
15

To determine the number of T cells, use
rosette formation method with red blood cells
ram.
The method is based on the affinity of the CD2 receptor with
sheep erythrocyte membrane proteins. At
mixing lymphocytes with sheep erythrocytes
figures in the form of rosettes are formed.
Number of rosette-forming cells (E-ROC)
corresponds to the number of T-lymphocytes (CD2+
cells).
To determine the number of B cells, use
EAC sockets. Lymphocytes are mixed with
bovine red blood cells treated
complement and antibodies to red blood cells.
The modern method is flow cytometry.
16

It is of utmost importance
calculation of immunoregulatory
CD4/CD8 index (helper-suppressor ratio).
CD8+ are carried by T-suppressor and Tkiller cells, part of NK-cells.
CD4+ are carried by T-helpers and Tinductors, monocytes, T-cells of the DTH.
17

18

Basic principle of immunocytometry:

Fluorescent labeled mAbs
the cell under study passes with
flow of liquid through the capillary.
The flow is crossed by a laser beam.
The device records the reflection from
cell surface signal
"yes/no" principle.
By changing the transmitted laser
wave parameters are determined and
cage dimensions (straight and lateral
light scattering).
The laser beam induces
fluorescence of MCA on the surface
cells, which provides information about
the presence of certain receptors
structures.
As a result of the summation
information on the entire population
cells the device produces accurate
quantitative and qualitative
analysis of cellular state
populations.
19

The standard MCA panel allows you to determine
the following DM markers: DM3 (T-cells), DM4 (T-helpers), DM8 (T-cytotoxic), DM20 (B-cells),
CD16 (NK cells), CD14 (monocytes/macrophages), CD25
(IL-2 receptor).
20

Methods for studying the main
components of the immune system accepted
also divided into screening and
expanded.
When assessing the B-system of immunity to
screening tests include determining
number of CD19+ and CD20+ cells, IgG, IgM and IgA,
to deployed - blast transformation
(RBTL) for the mitogen of milkweed and S.aureus,
surface markers of B lymphocytes.
21

Immunoglobulins Jg

Immunoglobulin A. Normal: 0.6–4.5 g/l.
JgA increases during acute infections, autoimmune
diseases (usually in the lungs or intestines), nephropathies.
A decrease in JgA occurs in chronic diseases (especially
respiratory system and gastrointestinal tract), purulent
processes, tuberculosis, tumors, immunodeficiencies.
Immunoglobulin E. Normal: 0-0.38 mg/l. The quantity is increasing
JgE for hereditary allergic reactions,
allergic lesions of the respiratory system by fungus
Aspergillus, helminthic infestation
A decrease in JgE occurs with chronic infections, taking
drugs that inhibit cell division, innate
immunodeficiency diseases.
22

Immunoglobulin M. Normal: 0.6–3.4 g/l.
The JgM content increases with
bronchial asthma, infections (acute and
chronic), during exacerbations, autoimmune
diseases (especially rheumatoid
arthritis). JgM decreases during primary and
secondary immunodeficiencies.
Immunoglobulin G. Normal: 6.0-17.6 g/l.
The amount of JgG increases in the blood when
allergies, autoimmune diseases,
past infections.
A decrease in JgG content occurs when
primary and secondary immunodeficiencies.
23

Second level tests - a more in-depth analysis of the state of the immune system
carried out using analytical methods: assessment methods
functional activity of T- and B-lymphocytes, phagocytes,
auxiliary cells, natural killer cells, system components
complement, etc.
immunophenotyping tests to determine relative and
absolute number of populations and subpopulations of T-, B-, NK-lymphocytes;
lymphocyte activation markers;
assessment of the various stages of phagocytosis and the receptor apparatus
phagocytic cells;
determination of the main classes and subclasses of immunoglobulins;
circulating immune complexes;
determination of the concentration of complement components in blood serum
(C3, C4, C5, C1-inhibitor);
functional activity of various subpopulations of lymphocytes;
assessment of the proliferative activity of T- and B-lymphocytes;
study of interferon status;
skin tests etc.
24

All of the above standards
indicators of immune status can
vary slightly in different
immunological laboratories. This
depends on the diagnostic technique and
reagents used. But immune
system, like any other system
body, may have disorders in
any links. This is how they arise
immunodeficiencies.
25

It should be especially emphasized that a full analysis
immunograms are possible only in combination with clinical
condition and medical history of the patient.
Absence of characteristic changes in the immunogram during
expressed clinical symptoms should be considered
an atypical reaction of the immune system, which is
aggravating sign of the disease.
The obtained patient data are compared with the average
values ​​for a given analyte obtained in the region
patient's residence. Average indicators
vary depending on the region and are subject to
climatic and geographical conditions, environmental conditions,
living conditions.
It is also necessary to take into account the patient's age and circadian
rhythms.

Violation of the mechanisms of the immune response leads to various pathologies of the immune system that are dangerous to health and life. The most common form of this pathology is immunological deficiency, or, according to generally accepted international terminology, immunodeficiency states. Let us briefly consider the general patterns of functioning of the immune system.

First, the effectiveness of the immune system is based on the balance of its components. Each component of the immune system largely replicates the functions of the other components. Thus, a defect in some components (or links) of the immune system can often be compensated by other components of the immune system. Therefore, if a person has a defect in any immune component, it is necessary to use drugs that improve cell metabolism as an adjuvant.

Secondly, the cells of the immune system carry out their basic functions in an active state. The main stimulus for the activation of all cells of the immune system is antigen. But there are situations when the antigen acts as a suppressive factor. For example, the phenomenon of so-called lazy leukocytes, which do not react actively enough to a foreign substrate, is known.

Thus, the immune status determines the individual reactivity of the body and reflects those boundaries of interaction with the environment, beyond which a normal reaction turns into a pathological one. Any acute illness is not a consequence of the fact that there are all kinds of pathogenic bacteria in the human environment. If this were so, then people would get sick all the time. But only those who react to a certain type of bacteria that is pathological for them get sick. Based on this, we can talk about three levels of the body’s reactivity, such as tolerance, resistance and immunity. A tolerant organism has no protection against pathological factors. Lack of protection leads to destruction of the body and death. This occurs in immunodeficiencies. A resistant organism, when encountering a pathological agent, reacts by turning on the immune system to fight it. The result of this fight will depend on the strength of the defense mechanisms of the quantity and quality of the pathogen. This struggle manifests itself as a pathological process. Immune organism interacts with the pathogen, and the result of its reaction is the destruction of the pathogen at the level of normal body defense. But such a division is very conditional and relative. For example, an organism tolerant to one antigen may be resistant to another and immune to a third. In addition, there are intermediate types of reactions. This applies to chronic diseases when the immune defenses cannot completely destroy the antigen, but at the same time do not provide it with the opportunity to destroy the diseased organ or tissue. This struggle is going on with varying degrees of success, i.e. periods of remission (recovery) are replaced by periods of exacerbation of a chronic disease. When the body’s defense is insufficient, caused by a defect in any of the defense elements or weakness of the body itself, compensatory reactions generalize.

Thus, increasingly higher levels of the body, including vital systems, are involved in the fight against the pathogen. In this case, the body works to the limit. Compensatory reactions can reach such strength that life support systems begin to be affected. For example, during fever, body temperature as a result of thermal reactions can exceed the permissible level and cause death. In this case, death is the price of adaptation. This is just an isolated example, but it also shows how important it is for the body to have a good immunological status.

The study of immune status includes:

1) determination of blood group and Rh factor;

2) a general blood test with a detailed leukogram or formula;

3) determination of the amount of immunoglobulins;

4) study of lymphocytes;

5) study of the phagocytic activity of neutrophils.

In addition, there are two stages of immunological diagnostics. The first stage identifies “gross” defects in the immune system. Research is carried out using simple, so-called indicative methods. These are the first level tests. Therefore, the method is determined by twenty indicators: the number of leukocytes, lymphocytes, various subgroups of T-lymphocytes, the levels of immunoglobulins (Jg) A, M, J, E, the concentration of circulating immune complexes, etc. At this stage, the number of cells, their percentage and functional activity are taken into account . At the second stage, a more thorough analysis of the state of immunity is carried out if deviations in orientation tests. Second-level tests allow you to track changes in the content of complex substances involved in the regulation of the immune response (for example, interleukin), as well as the number of cells carrying a certain type of immunoglobulin. Analysis of immune status indicators is carried out over the course of the disease, so these studies must be repeated. This makes it possible to identify the nature and level of disorders and track their changes during the treatment process. It is necessary to dwell in more detail on deciphering the immunogram indicators.

1. Immune status

Leukocytes

Normal – 3.5–8.8 4 ? 10 9 /l. An increase in the number of leukocytes is leukocytosis, a decrease is leukopenia. Leukocytosis is divided into physiological and pathological. The causes of physiological leukocytosis can be food intake (with the number of leukocytes not exceeding 10–12 × 10 9 /l), physical work, taking hot and cold baths, pregnancy, childbirth, and the premenstrual period. For this reason, blood should be donated on an empty stomach and not before doing heavy work. physical work. For pregnant women, women in labor, and children, their own standards have been established. Pathological leukocytosis occurs in infectious diseases (pneumonia, meningitis, general sepsis, etc.), infectious diseases with damage to cells of the immune system (infectious mononucleosis and infectious lymphocytosis), various inflammatory diseases caused by microorganisms (furunculosis, erysipelas, peritonitis, etc. .). But there are also exceptions. For example, some infectious diseases occur with leukopenia (typhoid fever, brucellosis, malaria, rubella, measles, influenza, viral hepatitis in the acute phase). The absence of leukocytosis in the acute phase of an infectious disease is an unfavorable sign, which indicates a weak body resistance. The basis of inflammatory diseases of non-microbial etiology, the so-called autoimmune diseases (systemic lupus erythematosus, rheumatoid arthritis, etc.), infarctions of various organs, is non-microbial inflammation (necrosis); extensive burns, large blood loss.

Causes of leukopenia:

1) exposure to certain chemicals (for example, benzene);

2) taking certain medications (butadione, reopirin, sulfonamides, cytostatics, etc.);

3) radiation, x-rays;

4) disorder of hematopoiesis;

5) blood diseases (leukemia) – leukopenic and aleukopenic forms;

6) overdose of cytostatics during chemotherapy;

7) metastases of tumors to the bone marrow;

8) diseases of the spleen, lymphogranulomatosis;

9) some endocrine diseases (acromegaly, Cushing's disease and syndrome, some infectious diseases mentioned above).

Lymphocytes

Norm: absolute content – ​​1.2–3.0 ? 10 9 /l, but more often in a clinical blood test the percentage of lymphocytes is indicated. This figure is 19–37%. There are also lymphocytosis and lymphopenia. Lymphocytosis is detected in chronic lymphocytic leukemia, chronic radiation sickness, bronchial asthma, thyrotoxicosis, some infectious diseases (whooping cough, tuberculosis), and when the spleen is removed. Lymphopenia is caused by abnormalities in the development of the lymphoid system, ionizing radiation, autoimmune diseases (systemic lupus erythematosus), endocrine diseases (Cushing's disease, taking hormonal drugs), AIDS.

T lymphocytes

Norm: relative content 50–90%, absolute – 0.8–2.5? 10 9 /l. The number of T-lymphocytes increases in allergic diseases, during the recovery period, and in tuberculosis. A decrease in the content of T-lymphocytes occurs with chronic infections, immunodeficiencies, tumors, stress, injuries, burns, some forms of allergies, and heart attack.

T helper cells

Norm: relative content – ​​30–50%, absolute – 0.6–1.6 ? 10 9 /l. The content of T-helper cells increases during infections, allergic diseases, autoimmune diseases (rheumatoid arthritis, etc.). A decrease in the content of T-helper cells occurs in immunodeficiency states, AIDS, and cytomegalovirus infection.

B lymphocytes

Norm: relative content – ​​10–30%, absolute – 0.1–0.9? 10 9 /l. Increased levels occur during infections, autoimmune diseases, allergies, and lymphocytic leukemia.

A decrease in the number of B lymphocytes is found in immunodeficiencies and tumors.

Phagocytes (neutrophils)

Their activity is assessed using methods that determine the part of cells capable of forming a phagosome (digestive vesicle) inside themselves. To assess the digestive ability of neutrophils, the NBT test is used (NBT is a nitroblue tetrazolium dye). The norm of the NST test is 10–30%. The phagocytic activity of leukocytes increases in acute bacterial infections and decreases in congenital immunodeficiencies, chronic infections, autoimmune diseases, allergies, viral infections, and AIDS. The activity of phagocytes, i.e. “eater” cells, is assessed by the so-called phagocytic number (normally a cell absorbs 5-10 microbial particles), phagocytic capacity of the blood, number of active phagocytes, phagocytosis completion index (must be greater than 1.0) .

Immunoglobulins Jg (antibodies)

Immunoglobulin A. Normal: 0.6–4.5 g/l. JgA increases in acute infections, autoimmune diseases (usually in the lungs or intestines), and nephropathies. A decrease in JgA occurs in chronic diseases (especially the respiratory system and gastrointestinal tract), purulent processes, tuberculosis, tumors, and immunodeficiencies.

Immunoglobulin M. Normal: 0.4–2.4 g/l. The JgM content increases during bronchial asthma, infections (acute and chronic), during exacerbations, autoimmune diseases (especially rheumatoid arthritis). JgM decreases in primary and secondary immunodeficiencies.

Immunoglobulin J. Norm: 6.0-20.0 g/l. The amount of JgJ increases in the blood with allergies, autoimmune diseases, and past infections. A decrease in JgJ content occurs in primary and secondary immunodeficiencies.

When studying the immune status, the number of immune complexes (IC) is also determined. The immune complex consists of an antigen, antibody and related components. The content of IC in blood serum normally ranges from 30 to 90 IU/ml. The content of immune complexes increases during acute and chronic infections and makes it possible to distinguish these stages from each other, during allergic reactions (and determines the type of these reactions), during intoxication of the body (kidney diseases, immunoconflict), during pregnancy, etc.

All of the above norms for indicators of immune status may differ slightly in different immunological laboratories. This depends on the diagnostic technique and the reagents used. Normal indicators of immune status indicate a reliable “shield” of the body and, therefore, that a person is in good health. But the immune system, like any other system of the body, can have disorders at any level. In other words, the immune system itself may be “sick.” So-called immunodeficiencies occur. The basis of immunodeficiency states are violations of the genetic code that do not allow the immune system to carry out one or another part of the immune response. Immunodeficiency conditions can be primary or secondary. In turn, primary ones are congenital, and secondary ones are acquired.

2. Congenital immunodeficiencies

This pathology is genetically determined. Most often, congenital immunodeficiencies appear in the first months of life. Children very often suffer from infectious diseases, which often occur with complications. There is a working classification of congenital conditions of immune deficiency, proposed by WHO experts in 1971. According to this classification, primary immunodeficiencies are divided into five large groups.

The first group includes diseases that are associated only with a defect in B cells: sex-linked Bruton agammaglobulinemia, transient (transient) hypogammaglobulinemia, X-linked immune deficiency and hyperimmunoglobulinemia M, etc.

The second group includes diseases of immune deficiency with a defect of only T cells: hypoplasia of the thymus gland (DiGeorge syndrome), episodic lymphocytopenia, etc.

The third group is diseases with simultaneous damage to B and T cells: immune deficiency with or without hypergammaglobulinemia, immune deficiency with ataxia, telangiectasia (Louis-Barr syndrome), thrombocytopenia and eczema (Wiskott-Aldridge syndrome), thymoma (tumor of the thymus ) and etc.

The fourth group includes states of immunodeficiency in which B and T stem cells are simultaneously affected: immune deficiency with generalized hypoplasia of the hematopoietic system, severe, combined immune deficiency linked to the X chromosome, etc.

The final fifth group includes conditions of immune deficiency that are not qualified above.

In practice, congenital immune deficiency conditions are limited to three main groups:

1) defects in phagocytosis;

2) insufficiency of cellular and humoral immunity (T-, B- and stem cells);

3) dysfunction of the complementary system.

Defects in phagocytosis constitute a large group of diseases. Here there are mainly dysfunctions of granulocytes and related cells: chronic idiopathic neutrocytopenia with lymphocytosis (essential benign granulocytopenia, often affecting premature babies), autosomal recessively inherited agranulocytosis, which begins in early infancy and ends in the death of the child from bacterial infections in the first years his life, dysfunction of granulocytes, degranulation syndrome (congenital dysphagocytosis), congenital hypoplasia of the spleen, etc.

Defects in humoral and cellular immunity cause the following conditions:

1) severe combined immune defect syndrome with impaired cellular immunity and antibody formation;

2) thymic hypoplasia (DiGeorge syndrome);

3) absence of purine nucleoside phosphorylase;

4) ataxia-telangiectasia syndrome;

5) thymoma with immunodeficiency syndrome, etc.

The clinical manifestations of congenital immunodeficiency conditions are very diverse. They vary from severe symptoms caused by past infections or vaccinations, to moderate and mild recurrent and difficult to diagnose painful phenomena. Congenital or primary immunodeficiencies are one of the common reasons early childhood mortality. Patients with immune deficiency have a family history of severe recurrent inflammation of the skin, mucous membranes, respiratory and digestive tracts (otitis, bronchopneumonia, enteritis, pyoderma, candidiasis, sepsis, etc.). With a deficiency of B-lymphocytes, bacterial infections caused by pneumococci, streptococci, and meningococci develop. T-lymphocyte deficiency is characterized by viral, fungal and mycobacterial infections. In children with T-system deficiency, viral infections are severe. With immune deficiency, children find it difficult to tolerate antiviral and antibacterial vaccinations, even leading to death.

Deficiency of humoral immunity manifests itself in the second half of the year with bacterial infections. With a deficiency of cellular immunity, fungal and viral infections develop immediately after birth. Now about congenital immunodeficiency conditions in more detail.

Diseases of humoral immunity, X-linked agammaglobulinemia

This disease is based on an isolated defect of B lymphocytes that cannot mature into plasma cells, is inherited recessively, linked to the X chromosome, and is the first described state of immune deficiency. Only boys suffer from this disease. The body cannot produce all classes of immunoglobulins, and without treatment, children die at an early age from recurrent infections. In many cases, patients develop well until 6-8 months of age. This appears to be due to the transplacental transfer of immunoglobulins from the mother. Pathology manifests itself when the received reserves are completely exhausted. This is a relatively rare disease - approximately 13 patients per 1,000,000 boys.

Clinically, the disease manifests itself in the fact that boys often suffer from recurrent infections caused by pneumococci, streptococci, and influenza virus. Less common are infections caused by meningococci and staphylococci. The infectious process is localized in the paranasal sinuses, middle ear, bronchi, lungs, and in the membranes of the brain. In such patients, the course of viral infections is the same as in healthy children, with the exception of viral hepatitis and enteroviral infections. Affected boys do not have tonsils (tonsillar tissue) and lymph nodes. On laboratory examination, the lymphocyte count is usually normal. When determining B- and T-lymphocytes, a very pronounced decrease in the number of B-lymphocytes and a normal number of T-lymphocytes are detected.

Selective JgA deficiency

This is an isolated JgA deficiency with normal or elevated levels of other immunoglobulins. It is the most common immunodeficiency condition, found in healthy individuals in 1:300 to 1:3000 cases. various studies. The absence of JgA is quite often combined with chromosomal abnormalities (especially the 18th pair of chromosomes), with developmental defects after intrauterine infections. It is likely that the 18th pair of chromosomes contains a gene that regulates the synthesis of JgA... The clinical manifestations of this pathology are very diverse: from the complete absence of symptoms to severe diseases. The most commonly observed are pulmonary infections, diarrhea and autoimmune diseases. Damage to the digestive and respiratory systems is explained by the absence of the secretory component JgA... Patients with selective JgA deficiency have an increased tendency to form immune complexes. This explains the often observed selective JgA deficiency in systemic lupus erythematosus, rheumatoid arthritis, pernicious anemia, thyroiditis, diabetes mellitus, Addison's disease, chronic active hepatitis, etc.

Immune deficiency with increased JgM content

The disease is genetically determined, inherited recessively, transmitted on the X chromosome and is characterized by an increase in JgM with normal or decreased levels of JgJ and JgA in the blood plasma. There is another name for this immunodeficiency - dysgammaglobulinemia I and II.

Clinical signs appear in the first or second year of life in the form of severe, frequently recurring bacterial infections. The most common purulent infections are: skin abscesses, oral ulcerations, otitis, tonsillitis, lymphadenitis, sinusitis, respiratory tract lesions. Sometimes the disease generalizes and leads to sepsis. Patients with hyperimmunoglobulinemia M often develop autoimmune diseases. The disease is complicated by neutropenia.

Transient hypogammaglobulinemia in infants

It is known that only JgJ class antibodies pass into the placenta. After incomplete breakdown of immunoglobulins, antibodies accumulate in the placenta. Having penetrated the fruit in this form, they are again resynthesized into whole JgJ molecules. As a result, some newborns may have levels of JgJ in their blood that are higher than their levels in their mother's blood. The mother's antibodies and the baby's immunoglobulins are usually metabolized after birth, and the concentration of JgJ begins to decrease, reaching its minimum between the 3rd and 6th months of life.

Clinically, these changes are manifested by low resistance to infections in the second half of the child’s life. Healthy infants can overcome this physiological hypogammaglobulinemia because immediately after birth the baby is exposed to antigens that trigger its own production of immunoglobulins. The JgM system is the first to be activated, as a result of which antibodies of this system are detected in the blood a few days after birth. JgJ reacts more slowly - within several weeks, and the concentration of JgA reaches their values ​​in adults only after several months or even years. Secretory JgA is formed in large quantities in a much shorter time. Activation of the fetus’s own synthesis of immunoglobulins is possible with intense antigenic stimulation. In this case, the JgM system reacts especially quickly and intensely. Therefore, the detection of increased levels of JgM in the blood serum of newborns indicates the presence of intrauterine infection.

In infants, there are several types of transient (transient) hypogammaglobulinemia. The most common is physiological hypogammaglobulinemia, which usually disappears by the end of the first six months of the child’s life. Pathological hypogammaglobulinemia is observed in premature infants, since the transfer of immunoglobulins across the placenta begins by the end of the 20th week and continues until birth. There is a clear relationship between gestational age and immunoglobulin levels. Their low value is influenced by the limited ability to synthesize immunoglobulins in premature infants. Also, pathological hypogammaglobulinemia in infants can be observed with maternal hypogammaglobulinemia, which is compensated under the influence of their own products. And finally, pathological transient hypogammaglobulinemia occurs in cases of delayed maturation of the immunoglobulin production system. This may be due to lack of contact with antigens, as well as unknown reasons. The diagnosis of transient hypogammaglobulinemia in infants is made on the basis of low immunoglobulin levels and the ability to form antibodies after vaccinations, which is not observed with persistent (aggressive) hypogammaglobulinemia.

X-linked immunoproliferative disease

This disease is manifested by immune deficiency and an increased susceptibility to lymphoma. The syndrome is named after the first described family - Duncan disease. In this family, three brothers died of infectious mononucleosis, and four male relatives of the mother had lymphoma and unusual complications of infectious mononucleosis in the form of immunoblastic sarcoma, hypogammaglobulinemia, and immune deficiency with hypergammaglobulinemia M. Subsequently, this disease was described in other families.

Most of the patients had clinical and laboratory signs of long-term infectious mononucleosis. At the same time, patients had rapidly progressing and fatal diseases with pathological proliferation of lymphoid tissue, such as plasmacytoma, African Burkitt lymphoma, B-cell immunoblastic sarcoma, and histiocytic lymphoma.

3. Diseases of cellular immunity

These diseases are rare due to their severe course and fatal outcomes in early childhood.

Children with partial or complete T-lymphocyte deficiency most often suffer from severe infections that cannot be treated. In these conditions, the level of serum immunoglobulins is either normal or elevated. Of this group, the main ones are two syndromes: DiGeorge syndrome (thymic hypoplasia) and cellular immunodeficiency syndrome with immunoglobulins.

Thymic hypoplasia (DiGeorge syndrome)

With this syndrome, embryonic cells from which the parathyroid glands and thymus develop are affected in utero. As a result, the parathyroid glands and thymus are either underdeveloped or completely absent in the child. The tissues from which the face is formed are also affected. This is expressed by underdevelopment of the lower jaw, short upper lip, characteristic palpebral fissures, low location and deformation of the ears. In addition, children have congenital disorders of the heart and large vessels. The disease appears sporadically, but there are suggestions that it is genetically determined and inherited in an autosomal recessive manner.

Clinically, DiGeorge syndrome manifests itself at birth. Facial disproportions and heart defects are characteristic. The most characteristic symptom during the newborn period is hypocalcemic seizures (due to underdevelopment of the parathyroid glands). Immunodeficiency syndrome develops more often in the second half of the life of an infant and is clinically manifested by frequently recurring infections caused by viruses, fungi and opportunistic bacteria, up to severe septic processes. Depending on the degree of underdevelopment of the thymus gland, the symptoms of immune deficiency can be very different (from severe to mild), and therefore in mild cases they speak of partial DiGeorge syndrome. The blood shows low levels of calcium and high levels of phosphorus and a decrease or complete absence of parathyroid hormone, which confirms the underdevelopment or absence of the parathyroid glands.

Severe combined immunodeficiency conditions

A group of immune system diseases has been identified, called severe combined immunodeficiency conditions. Enzyme defects have been identified in the pathogenesis. Such immunodeficiencies are relatively rare diseases. Occurs in cases ranging from 1:20,000 to 1:100,000 in newborns. Despite the similar clinical picture, severe combined immunodeficiencies are divided into several subgroups based on pathogenetic and pathophysiological principles.

Swiss type (lymphoid stem cell type)

In most cases it is hereditary. Inheritance can be either X-linked recessive or autosomal recessive. In these diseases, the reproduction and differentiation of B-lymphocytes and T-lymphocytes are impaired. A decrease in the concentration of T cells and immunoglobulins (antibodies) in the blood is characteristic. Often this pathology is accompanied by other developmental defects.

Adenosine deaminase deficiency

In severe combined immunodeficiency, approximately 1/3 and 1/2 of patients have a deficiency of the enzyme adenosine deaminase. A deficiency of this enzyme leads to the accumulation of adenosine monophosphate, which in high concentrations is toxic to lymphocytes. Manifestations of the disease are typical for patients with severe combined immunodeficiency, but in approximately 50% of cases, abnormalities of cartilage tissue are also observed. Previously, these patients were classified as immune deficient with short stature and short limbs. Severe leukopenia is detected in the blood, as well as the absence of granulocytes and their precursors in the bone marrow. There are no JgA and JgM in the blood, and the amount of JgJ corresponds to the values ​​of JgJ that entered the child’s body through the placenta from the mother.

The main clinical symptom of this group of diseases is a pronounced tendency to infectious diseases, which appear from the first month of a child’s life and are most often extensive: all contact surfaces of the body are affected (skin, digestive system, respiratory tract). Pyoderma, abscesses and various types of rashes are observed. Lesions of the gastrointestinal tract manifest themselves in the form of repeated, untreatable diarrhea, which causes severe malnutrition. Respiratory tract infections are complicated by a deep, dry, whooping cough and pneumonia. Children often have prolonged hyperthermia, which is an expression of hematogenous sepsis or meningitis. In such conditions, infectious processes are caused by a variety of microorganisms: saprophytic bacteria and bacteria that cause purulent inflammation, viruses, protozoal pathogens and fungi. Laboratory tests reveal severe lymphopenia. The number of B- and T-cells in the blood is significantly reduced, and the thymus gland is not detected on x-ray. Typically, the clinical manifestations appear after the third month of the child’s life, i.e., when the JgJ transferred from the mother’s body through the placenta before birth is exhausted. Hemagglutinins and specific antibodies are not detected in the blood after immunizations. Cellular immunity is significantly impaired. In such patients, the nodes are very small with structural changes; severe atrophy is observed in the intestinal mucosa lymphatic system. If the thymus gland is detected, then very characteristic changes morphology, structural abnormalities, severe lymphopenia, absence of Hassall bodies.

4. Partial combined immunodeficiency conditions

Immune deficiency with thrombocytopenia and eczema (Wiskott-Aldrich syndrome)

This syndrome is characterized by a triad: thrombocytopenia, eczema and an increased susceptibility to infectious diseases.

It is inherited recessively, transmitted on the X chromosome, and is relatively rare.

Clinically, this disease manifests itself very early, already in the neonatal period. Children have skin hemorrhages, mainly petechial, and bloody diarrhea. In a later period, nosebleeds appear. Hemorrhages can be fatal. In the first three months of life, eczema appears, often complicated by hemorrhages. There may be other manifestations of allergies with high eosinophilia. In the first half of the child’s life, during the course of the disease, severe respiratory tract infections, complicated eczema, meningitis, and sepsis appear. With age, immune deficiency deepens and becomes aggravated. The most common causative agents of infections are pneumococci, which cause recurrent pneumonia, otitis, meningitis and sepsis. These diseases occur in early infancy. When cellular immunity is already affected, diseases can be caused by fungi and viruses. Of interest is the fact that with Wiskott–Aldrich syndrome, a fairly high risk of malignant tumors has been identified, amounting to 10–15%.

Ataxia, telangiectasia (Louis-Barr syndrome)

Louis-Barr syndrome is a complex disease of the immune, nervous and endocrine systems, with frequent involvement of the skin and liver. The disease is inherited through a pathological autosomal recessive gene.

A characteristic symptom of the disease is progressive cerebral ataxia, which usually appears at school age in children who were healthy before this age. At the age of three to six years, telangiectasia (changes in blood vessels) is established. The conjunctivae are most often affected (small veins are greatly dilated and tortuous). Such expansions are observed in ears and on the cheeks. At the same time, the skin looks prematurely aged, and graying of hair during puberty is common. In 80% of cases, patients are prone to infections that mainly affect the respiratory tract. Generalization infectious process and no damage to the digestive system is observed.

In addition to the main symptoms, there are also endocrinological abnormalities (sexual dysfunction, short stature, glucose intolerance, insulin-resistant diabetes mellitus) and liver function disorders. Patients have a tendency to malignant diseases of the lymphoreticular type. In this disease, a common immunological abnormality is selective deficiency of JgA, while JgJ values ​​are normal or slightly decreased, and JgM concentrations are normal or increased. JgE levels are usually low. Most patients have signs of impaired cellular immunity. The total number of lymphocytes was slightly reduced, and the number of circulating T-lymphocytes was significantly reduced.

Chronic granulomatous disease

This pathology is referred to as congenital diseases immunity associated with disorders of the phagocytic function of neutrophil leukocytes. In this disease, granulocytes are unable to destroy microorganisms. It is relatively rare. It can be inherited through a recessive, X-linked pathological gene or through an autosomal recessive gene.

Clinically manifested by numerous recurrent infections that appear at the most early period life. The skin most often affected is where small abscesses first appear, which quickly penetrate into the underlying tissue and are very difficult to cure. The majority have lesions of the lymph nodes (especially the cervical ones) with the formation of abscesses. Cervical fistulas also often appear. The lungs may be affected, which is manifested by recurring pneumonia, the digestive system in the form of inflammatory processes in the esophagus, liver, and also in the mediastinum.

In the blood, pronounced leukocytosis with a shift to the left, an increase in ESR, hypergammaglobulinemia, and anemia are detected. The prognosis for chronic granulomatous disease is poor. Most patients die in preschool age.

Immunodeficiency with complement deficiency

Complement refers to humoral immunity (from the Latin gumor - “liquid”). This is a group of proteins circulating in the blood serum that prepare bacteria and their toxins for phagocytosis and are also capable of directly destroying microorganisms. An insufficient amount of complement leads to the fact that the body has great difficulty fighting microbes, and this leads to the development of severe infectious diseases (including sepsis).

In some diseases, such as systemic lupus erythematosus, secondary complement deficiency may develop.

5. Acquired immunodeficiencies

They are also called secondary immunodeficiencies, since they appear during a person’s life for a variety of reasons. In other words, they arise as a result of the influence of many damaging factors on an organism that at birth had a healthy immune system. These damaging factors may be:

1) unfavorable ecology (water, air pollution, etc.);

2) nutritional disorders (irrational diets causing metabolic disorders, starvation);

3) chronic diseases;

4) prolonged stress;

5) incompletely cured acute bacterial and viral infections;

6) diseases of the liver and kidneys (organs that provide detoxification of the body);

7) radiation;

8) incorrectly selected medications.

Scientific and technological progress has led our civilization to the use of a huge number of artificial (synthetic) additives in food, medicines, hygiene products, etc. If these factors affect the body for a long time, then toxic products and metabolic products accumulate in the blood and lymph in such a way concentrations that chronic diseases develop. As a result, some types of bacteria that were absorbed by macrophages (phagocytes) do not die, but begin to actively multiply, which leads to the death of the phagocyte. Under normal conditions, microorganisms should die. The problem of secondary immunodeficiencies is very relevant for our time. They can seriously change and aggravate diseases, influence their outcome and the effectiveness of treatment.

There are temporary immunity disorders, so-called functional disorders. They respond well to correction (most often in children). A temporary decrease in the activity of immune parameters can also occur in healthy people. This is usually associated with seasonal phenomena (decrease in solar activity, humid weather), which leads to epidemic outbreaks of colds and flu. With timely detection, functional changes in immunity are easily restored to normal. If secondary immunodeficiencies disrupt the body’s self-cleansing processes, then over time this imbalance can lead to autoimmune diseases, oncology, and AIDS. All these types of secondary immunodeficiency conditions are quite serious diseases, have severe clinical manifestations and often unfavorable prognosis and outcome.

Autoimmune diseases

These diseases can occur when exposed to unfavorable environmental factors. The pathogenesis of autoimmune pathologies is based on disruption of the functioning of T-lymphocytes (suppressors). As a result, the immune system begins to show aggression against its own (healthy) cells of its own body. “Self-damage” of tissues or organs occurs.

Autoimmune diseases have a hereditary predisposition. These diseases include rheumatoid arthritis, systemic lupus erythematosus, periarthritis nodosa, scleroderma, systemic vasculitis, dermatomyositis, rheumatism, ankylosing spondylitis (ankylosing spondylitis), some diseases of the nervous system (for example, multiple sclerosis), etc. All autoimmune diseases have development according to the principle of a vicious circle. Schematically, this circle can be described as follows. When foreign agents (bacteria, viruses, fungus) invade a cell, an inflammatory reaction develops with the goal of isolating and rejecting the harmful agent. At the same time, the body’s own tissue changes, dies and becomes foreign to the body, and the production of antibodies begins against it, as a result of which inflammation develops again. When it reaches the stage of necrosis, the necrotic tissue also becomes an antigen, a harmful agent, to which antibodies are again produced, resulting in inflammation again. Antibodies and inflammation destroy this tissue. And this happens endlessly, a painful and destructive circle is formed. The primary agent (bacteria, virus, fungus) is no longer there, and the disease continues to destroy the body. The group of autoimmune diseases is quite large, and studying the mechanisms of development of these diseases is of great importance for developing tactics for their treatment and prevention, since most of these diseases lead to disability in patients.

A particularly significant share among autoimmune diseases is occupied by collagenosis, vasculitis, rheumatic lesions of the joints, heart, and nervous system.

Rheumatoid arthritis

This is a systemic connective tissue disease that manifests itself mainly as progressive inflammation of the joints. The causes are little known. The immunogenetic theory is considered the most likely. It suggests the presence of a genetically determined defect in the immune system. The mechanism of disease development is associated with autoimmune disorders. The main disorders concern the so-called rheumatoid factors, which are antibodies to immunoglobulins. Immune complex processes lead to the development of synovitis, and in some cases to generalized vasculitis. Granulation tissue forms and grows in the synovial membrane, which over time destroys cartilage and other parts of bones with the occurrence of erosions (usur). Sclerotic changes develop, fibrous and then bone ankylosis occurs (the joint becomes deformed and becomes stiff). Pathological changes occur in the tendons, serous bursae and joint capsule.

Clinically, the disease manifests itself as persistent inflammation of the joint (arthritis). But the most common is polyarthritis, affecting mainly small joints (metacarpophalangeal, interphalangeal and metatarsophalangeal). There are all signs of inflammation (pain, swelling of the joints, local fever). The disease is characterized by a gradual, slow, but steady progression of arthritis and the involvement of more and more joints in the pathological process. The advanced stage of the disease is characterized by deforming arthritis. Particularly typical are deformities of the metacarpophalangeal (flexion contractures, subluxations) and proximal (distant) interphalangeal joints. These changes form the so-called rheumatoid hand and rheumatoid foot.

In rheumatoid arthritis, it is rare, but extra-articular manifestations are also observed. These include subcutaneous nodules, most often located in the area of ​​the elbow joints, serositis (inflammation in the pleura and pericardium), lymphadenopathy, and peripheral neuropathy. The severity of extra-articular manifestations is usually small. Usually they do not come to the fore in the overall picture of the disease. Approximately 10–15% of patients develop kidney damage in the form of amyloidosis with gradually increasing proteinuria, nephrotic syndrome, which ends in renal failure. Laboratory findings are nonspecific. In 70–80% of patients, rheumatoid factor is detected in the blood serum (Waaler-Rose reaction). This form of rheumatoid arthritis is called seropositive. From the very beginning of the disease there are increase in ESR, fibrinogen, ? 2-globulins, appearance C-reactive protein in blood serum, decrease in hemoglobin level. All these indicators usually correspond to disease activity.

Systemic vasculitis

This is a group of diseases in which systemic lesion vessels with an inflammatory reaction of the vascular wall. There are primary and secondary systemic vasculitis. In primary cases, systemic vascular damage is an independent disease, while secondary ones develop against the background of some infectious-allergic or other disease. Secondary systemic vasculitis in diseases such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, acquire critical importance in the clinical picture of these diseases.

Primary systemic vasculitis includes hemorrhagic vasculitis, giant cell temporal arteritis, Wegener's granulomatosis, thromboangiitis obliterans, Goodpasture, Moshkovich, and Takayasu syndromes.

Hemorrhagic vasculitis (capillary toxicosis, Henoch-Schönlein disease)

This is a systemic lesion of capillaries, arterioles, and venules. The process occurs mainly in the skin, joints, abdominal cavity, kidneys. The disease usually occurs in children and adolescents, less often in adults of both sexes. The development of the disease occurs after an infection (streptococcal tonsillitis or exacerbations of chronic tonsillitis or pharyngitis), as well as after vaccination, due to drug intolerance, hypothermia, etc.

Damage to blood vessels in the form of microthrombosis, hemorrhages (hemorrhages), changes in the inner lining of the artery (endothelium) is of immune origin. Damaging factors are immune complexes circulating in the blood.

Clinically, the disease manifests itself as a triad:

1) small-celled, sometimes merging hemorrhagic skin rashes (purpura);

2) pain in the joints or inflammation of the joints, mainly large ones;

3) abdominal syndrome (pain in the abdominal cavity).

Most often the rash occurs on the legs. Initially, skin rashes are located on the extensor surfaces of the extremities, sometimes on the torso, often ending in residual pigmentation. More than 2/3 of patients have migrating symmetrical polyarthritis, usually of large joints. Inflammation of the joints is often accompanied by hemorrhages inside the joint cavity, which leads to pain of various types: from slight aches to severe pain, even immobility. Abdominal syndrome is manifested by sudden intestinal colic, which simulates appendicitis, cholecystitis, pancreatitis. Often the kidneys are involved in the pathological process in the form of glomerulonephritis due to damage to the glomerular capillaries. There is an acute course of the disease with a sudden, violent onset, multi-symptom clinical picture, and frequent renal complications. In the chronic course, recurrent skin-articular syndrome is more often observed.

Wegener's granulomatosis

Granulomatous-necrotizing vasculitis with predominant damage to the respiratory tract, lungs and kidneys. The reason is not yet known. The disease is provoked by colds (ARVI), cooling, overheating in the sun, trauma, drug intolerance, etc. The leading mechanisms for the development of the disease are autoimmune.

The disease develops more often in men. First, the respiratory tract is affected, which manifests itself in two ways. In the first option, a persistent runny nose with serous-purulent discharge and nosebleeds are noted; in the second, a persistent cough with bloody-purulent sputum and chest pain. Next, the clinical picture develops with many syndromes. This is the stage of generalization, which is accompanied by fever, transient polyarthritis or only pain in the joints and muscles, skin lesions (up to severe necrotic lesions of the facial skin), etc. The most typical occurrence is purulent-necrotic and ulcerative-necrotic rhinitis, sinusitis, nasopharyngitis and laryngitis. Clinical and radiological symptoms of the lungs manifest themselves in the form of focal and confluent pneumonia with the formation of abscesses and cavities. At this stage, the kidneys, heart, nervous system, etc. are involved in the pathological process.

In blood tests, changes are not specific (clear signs of inflammation - leukocytosis, accelerated ESR). The prognosis of the disease is often unfavorable. Patients die from pulmonary-cardiac or renal failure, pulmonary hemorrhage. The diagnosis is made on the basis of a biopsy of the mucous membranes of the respiratory tract and lungs, where the granulomatous nature of the disease is revealed.

Giant cell arteritis (temporal arteritis)

This is a systemic disease primarily affecting the temporal and cranial arteries. A viral etiology is assumed, and the development mechanism (pathogenesis) is immune complex damage to the arteries, which is confirmed by the detection of fixed immune complexes in the arterial wall. The granulomatous type of cellular infiltrates is also characteristic. Elderly people of both sexes get sick. In the most common variant, the disease begins acutely, with high temperature, headaches in temporal region. There is a visible thickening of the affected temporal artery, its tortuosity and pain on palpation, and sometimes redness of the skin. When the diagnosis is made late, damage to the blood vessels of the eye and the development of partial or complete blindness are observed. From the first days of the disease, the general condition also suffers (lack of appetite, lethargy, weight loss, insomnia).

Blood tests reveal high leukocytosis, neutrophilia, accelerated ESR, hyper-? 2 and gammaglobulinemia. The course of the disease is progressive, but early treatment can lead to lasting improvement.

Goodpasture's syndrome

This is a systemic capillaritis with predominant damage to the lungs and kidneys in the form of hemorrhagic pneumonia (with hemorrhages in the lung tissue) and glomerulonephritis (damage to the renal glomeruli). Young men (20–30 years old) are more often affected. The reason is not clear, but a connection with a viral or bacterial infection or hypothermia is considered more likely. It is characteristic that this disease was first described during the influenza pandemic in 1919. The pathogenesis is autoimmune, since antibodies to the basement membranes of the kidneys and lungs are found circulating and fixed in the tissues. Electron microscopic examination reveals changes in the basement membranes of the alveoli of the lungs and renal capillaries in the form of fixation of antibodies to these basement membranes.

Clinically, the disease begins acutely, with high fever, hemoptysis or pulmonary hemorrhage, and shortness of breath. In the lungs, an abundance of moist rales are heard in the middle and lower sections, and on x-rays there are many focal or confluent darkening on both sides. Almost simultaneously, severe, rapidly progressing glomerulonephritis develops with nephrotic syndrome (edema, protein and blood in the urine) and the rapid development of renal failure. The prognosis is often unfavorable; patients die within the next six months or a year from the onset of the disease from pulmonary-cardiac and renal failure. Anemia, leukocytosis and accelerated ESR are detected in the blood. An immunological sign of the disease are antibodies to the basement membranes of the kidney.

Thrombotic thrombocytopenic purpura (Moschkowitz syndrome)

This is a systemic thrombotic microangiopathy, which is accompanied by thrombocytopenic purpura, intravascular coagulation (hemolysis), cerebral and renal symptoms. The cause and mechanism of development of the disease are not yet known. The immune nature of the disease is assumed. Mostly young women get sick. The disease begins suddenly, with an increase in temperature, the appearance of signs of intravascular coagulation, thrombocytopenic purpura and various neuropsychiatric disorders due to brain damage. Other organs are also affected, primarily the kidneys with rapid development of renal failure.

Clinically the disease manifests itself hemorrhagic syndrome, petechial (small cell) hemorrhages on the skin, nasal, gastric, gynecological, renal bleeding, hemorrhages in the fundus. Blood tests reveal anemia, reticulocytosis (immature blood cells), thrombocytopenia (lack of platelets), increased bilirubin and hypergammaglobulinemia. The course is steadily progressive with a rapid fatal outcome.

Takayasu syndrome (aortic arch syndrome, pulseless disease)

This syndrome is inflammatory process in the aortic arch (aortitis) and in the branches extending from it. In this case, their partial or complete obliteration develops. Other parts of the aorta may also be affected.

The causes (etiology) and mechanisms (pathogenesis) of this disease are not yet clear. The significance of immune disorders, which are based on genetic defects in the formation of the aortic wall, is assumed. Young women are more likely to get sick.

The syndrome manifests itself as a gradual increase in signs of circulatory disorders in the areas of the affected vessels. The main symptom is the absence of pulse in one or both arms, less often in the carotid, subclavian, and temporal arteries. Patients feel pain and numbness in the extremities, which intensify with physical activity, weakness in the arms, dizziness, often with loss of consciousness. When examining the eyes, cataracts and changes in the vessels of the fundus (narrowing, formation of arteriovenous anastomoses) are detected. Much less often, the coronary arteries are involved in the process with corresponding symptoms. When the abdominal aorta and renal vessels are damaged, vasorenal (renal) hypertension develops. The general signs of the disease include low-grade fever and asthenia. Laboratory indicators are moderate. The disease progresses slowly, with exacerbations in the form of ischemia of a particular area. Diagnosis can be made in the early stages using arteriography.

Thromboangiitis obliterans

This is a systemic inflammatory vascular disease with predominant damage to muscular arteries, as well as veins. The etiology and pathogenesis are not yet known. An allergic reaction to various external and internal environment body. Mostly men aged 30–45 years are affected. The disease begins gradually, with migratory thrombophlebitis, rapid fatigue and heaviness in the legs (primarily when walking in the area calf muscles), paresthesia (sensitivity disturbances). Later, intermittent claudication develops, pain in the legs persists even at rest, especially at night. There is a decrease in pulsation in the arteries of the lower extremities, which later disappears. Already in the early stages, trophic disorders appear on the affected limbs, which can turn into necrosis as a result of increasing ischemia. The disease can acquire the character of a systemic process with damage to the coronary, cerebral, mesenteric arteries with the development of ischemic phenomena according to the feeding zone of a particular artery. There is a deterioration in the general condition, subfebrile reactions, in particular accelerated ESR. The course is chronic, steadily progressing, with an increase in ischemic phenomena. With a systemic process, myocardial infarction, ischemic strokes, intestinal necrosis and other serious conditions are possible that worsen the prognosis.

Systemic lupus erythematosus

It is a chronic systemic autoimmune disease of connective tissue and blood vessels. This serious autoimmune disease is caused by a chronic viral infection. These are RNA viruses close to measles or measles-like viruses. The mechanism of development of the disease is quite complex. The body produces circulating autoantibodies, of which the most important diagnostic significance are antinuclear antibodies to the whole nucleus and its individual components, circulating immune complexes, primarily DNA antibodies to DNA complement, which are deposited on the basal membranes of various organs and cause their damage. with an inflammatory reaction.

This is the pathogenesis of nephritis, dermatitis, vasculitis, etc. Such a high reactivity of humoral immunity is explained by a decrease in control by T-lymphocytes, i.e., cellular immunity. Possible family genetic predisposition. Mostly teenage girls and young women are affected. The disease can be provoked by pregnancy, abortion, childbirth, the onset of menstruation, infections (especially in adolescents), prolonged exposure to the sun, vaccination, and the use of medications.

The disease has a gradual onset. Asthenia (weakness) and recurrent polyarthritis appear. Much less often there is an acute onset, characterized by fever, dermatitis, acute polyarthritis, and then a course with relapses and multi-syndromic symptoms. Multiple joint lesions (polyarthritis) and pain in them are the most common and early symptoms. The lesions mainly affect the small joints of the hands, wrists, and ankles, but the knee joints can also be affected. The severity and persistence of the lesion varies. A characteristic symptom of the disease is skin damage in the form of erythematous rashes on the face (redness) in the shape of a butterfly, i.e. on the bridge of the nose, cheeks and in the upper half of the chest in the form of a décolleté, as well as on the extremities. Almost all patients experience polyserositis in the form of pleurisy, pericarditis, perihepatitis, and perisplenitis. Dermatitis, polyarthritis and polyserositis are the diagnostic triad of systemic lupus erythematosus. Damage to the cardiovascular system is typical. Pericarditis usually develops, followed by myocarditis. Warty Libman–Sachs endocarditis is often observed with damage to the mitral, aortic and tricuspid valves. Vascular damage occurs in individual organs, but Raynaud's syndrome is possible, which appears long before the development of the typical picture of the disease.

Lung damage is associated with vascular-connective tissue syndrome that develops during the underlying disease and with secondary infection. The so-called lupus pneumonia is manifested by cough, shortness of breath, and soft, moist rales in the lower parts of the lungs. X-ray reveals strengthening and deformation of the pulmonary pattern due to the vascular component in the lower parts of the lungs, and sometimes focal-like shadows are detected. Pneumonia develops against the background of polyserositis, therefore, on x-rays, in addition to the main changes, a high position of the diaphragm with signs of adhesions and so-called linear shadows parallel to the diaphragm (disc-shaped seals) are detected lung tissue). The pathological process also affects the gastrointestinal tract. Anorexia, aphthous (ulcerative) stomatitis, and dyspepsia (digestive disorders) are noted. There may be abdominal pain syndrome, which is caused by involvement of the peritoneum in the process or vasculitis itself (damage to the mesenteric, splenic and other arteries). In the early stages of the disease, an enlarged liver is observed, although lupus hepatitis itself is extremely rare. As a rule, liver enlargement is caused by heart failure, pancarditis (damage to the pericardium, myocardium and endocardium) or severe effusion pericarditis. There may also be fatty liver degeneration.

A frequent and early sign of a systemic disease is an increase in all groups of lymph nodes and the spleen, which indicates damage to the reticuloendothelial system. 50% of patients develop lupus glomerulonephritis, so-called lupus nephritis. Its development usually occurs during the period of generalization of the process. Kidney damage in systemic lupus erythematosus has several variants: urinary, nephritic or nephrotic syndrome. In the diagnosis of lupus nephritis, intravital puncture biopsy with in-depth examination of the biopsy material (immunomorphological and electron microscopic) is of great importance. The combination of fever, recurrent articular syndrome and persistently accelerated ESR requires the exclusion of lupus nephritis. Observations show that almost every fifth patient with nephrotic syndrome has systemic lupus erythematosus.

Many patients in all phases of the disease experience damage to the neuropsychic sphere. At the initial stage of the disease, asthenovegetative syndrome is observed, and then signs of damage to all parts of the central and peripheral nervous system develop in the form of encephalitis, myelitis, and polyneuritis. Often there are combined lesions (systemic) of the nervous system in the form of meningoencephalo-, myelopolyradiculoneuritis. Laboratory data are of great diagnostic importance, especially with regard to the detection of a large number of LE cells (lupus cells, or lupus cells).

Specific for systemic lupus erythematosus are high titers of antibodies to DNA. In the case of acute (rapid) development of the disease, lupus nephritis is detected after 3–6 months, which occurs as a nephrotic syndrome. In the subacute course, a wave pattern is characteristic with the involvement of various organs and systems in the pathological process, which in the clinical picture manifests itself as polysyndromic. The chronic long course of the disease is characterized by relapses of polyarthritis and (or) polyserositis, Raynaud's syndrome and epileptiform seizures. Only in the 5th-10th year does the characteristic polysyndromy gradually develop. In accordance with the clinical and laboratory characteristics, three degrees of process activity are distinguished: high (III degree), moderate (II degree) and minimal (I degree). Patients require many years of continuous treatment. The best results are observed with early treatment, then stable clinical remission develops.

Dermatomyositis (polymyositis)

Refers to systemic diseases connective tissue with predominant damage to muscles and skin. It is assumed that the trigger for this disease is a viral infection, and the provoking factors are cold, injury, prolonged exposure to the sun, pregnancy, and drug intolerance. 20–30% of patients may have neoplastic dermatomyositis. The pathogenesis is based on autoimmune disorders. Neuroendocrine reactivity is important, since women predominate among patients (2:1), and the peak of the disease occurs in two age periods. These periods are puberty (the period of sexual development) and menopause, i.e., peaks of hormonal changes in the body. A family genetic predisposition is also possible.

The clinical onset of the disease can be either acute or gradual. Muscle syndrome comes to the fore in the form of muscle weakness and muscle pain (myasthenia gravis and myalgia). No less significant manifestations of the disease are arthralgia, fever, skin lesions, and dense widespread edema. Subsequently, the disease acquires a relapsing course. In all patients, skeletal muscles are affected. This is manifested by myalgia during movement and at rest, as well as with pressure, and is characterized by increasing muscle weakness.

The muscles of the shoulder and pelvic girdle become thicker and increase in volume, active movements are significantly impaired, to such an extent that patients cannot sit down independently, lift their limbs, lift their head from the pillow, or hold it while sitting or standing. If the process spreads significantly, the patients become immobilized, and in severe cases are in a state of complete prostration. If the pathological process spreads to the facial muscles, this leads to a mask-like appearance of the face, damage to the pharyngeal muscles leads to dysphagia, and damage to the intercostal muscles and diaphragm leads to respiratory failure, decreased ventilatory function of the lungs and, as a result, frequent pneumonia.

In the early stages of the disease, the muscles are painful and often swollen; later they undergo dystrophy and myolysis (resorption of muscle fibers). In even more late stages diseases, myofibrosis develops at the site of muscle fibers (replacement of muscle tissue with connective tissue), which leads to muscle atrophy and contractures. Calcification (calcium deposition) may occur in muscles and subcutaneous tissue, especially often in young people. Calcification is easily detected by X-ray examination. Electromyography changes are nonspecific. A variety of skin lesions are characteristic. These are all kinds of rashes in the form of reddened areas of the skin, the appearance of tubercles and blisters, dilation of skin vessels, keratinization of certain areas of the skin, depigmentation or hyperpigmentation, etc. Often these rashes are accompanied by itching. The presence of periorbital (around the eyes) edema with purplish-purple erythema - the so-called dermatomyositis spectacles - is very pathognomonic.

The joints are affected in the form of polyarthralgia (pain in many joints at once), up to the development of joint stiffness. There is inflammatory or dystrophic damage to the myocardium. With diffuse myocarditis, a severe picture of heart failure develops. Raynaud's syndrome is observed in 1/3 of patients. Lung damage due to hypoventilation is common. In almost half of the patients, the gastrointestinal tract is involved in the pathological process. This is manifested by anorexia, abdominal pain, gastroenterocolitis, and decreased tone of the upper third of the esophagus. Sometimes there are symptoms simulating intestinal obstruction. Laboratory findings are nonspecific. Usually this is moderate leukocytosis with pronounced eosinophilia (up to 25–70%), persistent moderate acceleration of ESR, hypergammaglobulinemia. Biochemical tests of blood and urine, muscle biopsy are important for diagnosis. Thickening of muscle fibers with loss of transverse striations, fragmentation and dystrophy, up to necrosis, accumulation of lymphocytes, plasma cells, etc. in the muscles are detected. In the acute course, a catastrophically increasing generalized lesion of the striated muscles is observed, up to complete immobility. Patients cannot swallow or speak. There is a general serious condition with fever, toxicosis and various skin rashes. If left untreated, death usually occurs within 3–6 months. The main causes of unfavorable outcome are aspiration pneumonia and pulmonary heart failure. The subacute course is marked by cyclicity, but there is also a steady increase in adynamia, damage to the skin and internal organs. The most favorable form is the chronic course of the disease, in which only individual muscles are affected, and patients remain able to work. The exception is young people who develop extensive calcifications in the skin, subcutaneous tissue, and muscles with the formation of persistent contractures and almost complete immobility.

Periarteritis nodosa

This is a systemic vascular disease with predominant damage to muscular arteries and smaller vessels. The disease occurs for an unknown reason. In pathogenesis, the main thing is the highest (hyperergic) reaction of the body in response to the influence of various factors. A significant role is played by immune complexes circulating and fixed in the vascular wall. Mostly men aged 30–40 years are affected.

The onset of the disease is acute or gradual, with such general symptoms as fever, progressive weight loss, pain in the joints, muscles, abdomen, skin rashes, and damage to the gastrointestinal tract. Over time, the heart, kidneys, and peripheral nervous system are affected, i.e., polyvisceral symptoms develop (all organs are affected). Almost all patients experience glomerulonephritis of varying severity: from mild nephropathy with transient (transient) hypertension and moderate urinary syndrome to diffuse glomerulonephritis with persistent hypertension and a rapidly progressive course. The unfavorable prognosis is the development of the syndrome of malignant hypertension and nephrotic syndrome, which quickly leads to renal failure. In addition, renal infarctions and aneurysms due to arteritis are observed. Almost 70% of patients have heart damage. Since the coronary arteries are affected, attacks of angina are observed up to the development of myocardial infarction, but without clear clinical signs. Sometimes an aneurysm and exudative (effusion) pericarditis are formed. Raynaud's syndrome may develop, which is rarely complicated by gangrene of the fingers. Migrating phlebitis (venous lesions) are sometimes observed.

Acute abdominal pain is very characteristic of periarteritis nodosa. They are associated with a pathological process in the vessels of the abdominal cavity. Damage to the vessels of the stomach leads to gastritis, damage to the vessels of the small intestine leads to enteritis, etc. Appendicitis may develop, acute cholecystitis, pancreatitis, intestinal perforation due to necrosis, infarction, hemorrhage. In 50% of patients, damage to the nervous system is manifested by multiple neuritis associated with pathology in the vessels supplying a particular nerve. Possible meningoencephalitis with speech and hearing impairment, headache and dizziness, convulsions, as well as focal brain damage due to thrombosis, ruptured aneurysms. One of the early symptoms of the disease is eye damage. When examining the fundus, arterial aneurysms, thrombosis of the central retinal artery, etc. are revealed.

Joint pain (arthralgia) is noted, and less commonly, arthritis of large joints, muscle pain, and various skin lesions. In a small group of patients, subcutaneous nodules, very characteristic of periarteritis nodosa, are found, which are vascular aneurysms or granuloma associated with the affected vessel.

A feature of periarteritis nodosa is the rapidly developing severe pallor of patients, which, in combination with exhaustion, creates a picture of chlorotic marasmus. Lung damage manifests itself as pneumonia and bronchial asthma. Pulmonary symptoms are associated with vascular damage. There are observations indicating that bronchial asthma may precede the full picture of periarteritis nodosa by many years.

Laboratory findings are unusual. Possible leukocytosis with a neutrophil shift, eosinophilia, sometimes high. In severe cases, moderate anemia and thrombocytopenia occur. To clarify the diagnosis, a muscle biopsy is performed from the area of ​​the legs or abdominal wall. In this case, vascular changes characteristic of this disease are revealed.

Rheumatism

Systemic inflammatory disease of connective tissue with predominant localization in the heart. Children and young people usually get sick. Women get sick approximately 3 times more often than men. The main cause of the disease is β-hemolytic streptococcus of group A. However, in patients with prolonged and continuously recurrent forms of heart damage of a rheumatic nature (rheumatic carditis), the connection of the disease with streptococcus is often not established, although the heart damage fully meets all the main criteria for rheumatism. This indicates other reasons for the development of rheumatism: allergic (not related to streptococcus or infectious antigens in general), infectious-toxic, viral.

Allergies play a significant role in the development of rheumatism. It is assumed that sensitizing agents (streptococcus, virus, nonspecific allergens, etc.) can initially lead to allergic inflammation in the heart, and then to a change in the antigenic properties of its components with their transformation into autoantigens and the development of an autoimmune process. Genetic predisposition plays an important role. Morphologically, the systemic inflammatory process in rheumatism manifests itself in characteristic phase changes in connective tissue. This is mucoid swelling - fibrinoid change - fibrinoid necrosis. Cellular reactions (infiltration of lymphocytes and plasma cells) also play an important role in the morphology of rheumatism. These cellular reactions are the histological reflection of allergies in rheumatism. From the stage of fibrinoid changes, complete tissue restoration is no longer possible; the process ends with sclerosis (i.e., replacement with connective tissue).

Clinical manifestations of the disease in typical cases develop 1–2 weeks after a sore throat or other infection. But with repeated attacks, this period may be shorter. In some patients, even primary rheumatism occurs 1–2 days after cooling without any connection with infection. Exacerbations develop after any concomitant diseases, operations, or physical exertion. Characteristic is the fact that the patient can clearly and accurately indicate the day of onset of the disease. In the first period of the disease, there is often an elevated temperature (usually subfebrile), the general condition remains unchanged. In some patients with polyarthritis or serositis, the condition can be severe: with high persistent fever up to 38–40 o C with daily fluctuations of 1–2 o C and heavy sweats(but no chills). However, in recent years this condition has been observed extremely rarely.

The most common manifestation of rheumatism is inflammatory damage to the heart. Any membranes of the heart can be involved in the process, but primarily the myocardium. It should be noted that rheumatism often occurs without any obvious changes in the heart. A pattern is noted: the older the patient who first fell ill with rheumatism, the less serious the rheumatic heart disease.

Rheumatic myocarditis. This disease in adults, as a rule, is not particularly severe. Patients complain of mild pain and vague discomfort in the heart area, slight shortness of breath during exercise, and, less often, sensations of palpitations or irregular heartbeats. On X-ray examination, the heart is of normal size or moderately enlarged. Circulatory failure practically does not develop. In some patients in childhood, the so-called diffuse rheumatic myocarditis may occur, which is manifested by violent allergic inflammation of the myocardium with severe swelling and dysfunction.

From the very beginning, the disease manifests itself as severe shortness of breath until a forced position is taken to facilitate breathing (orthopnea). Patients complain of constant pain in the heart area and rapid heartbeat. The so-called pale cyanosis and swelling of the neck veins are characteristic. The heart is significantly and evenly expanded. Very characteristic of diffuse myocarditis is the development of circulatory failure of both the left and right ventricular types. In adults, this variant of rheumatic myocarditis is currently practically never encountered.

Rheumatic endocarditis. It occurs in isolation and has very few general symptoms. The main signs of rheumatic endocarditis are systolic and diastolic murmurs, which appear to occur due to thrombotic deposits on inflammatory valves.

Occasionally, these overlays serve as sources of embolism in the vessels of the pulmonary or systemic circulation with the development of infarctions of the lungs, kidneys, spleen, gangrene of the extremities, central paralysis, etc. If endocarditis is the only localization of rheumatism, then the patients constitute the so-called outpatient group. This means that with this course of rheumatism, good general health and ability to work are maintained for a long time. After a certain time, a heart defect forms with accompanying hemodynamic disorders, and this forces patients to consult a doctor for the first time.

Pericarditis. For modern rheumatism it is very rare. Dry pericarditis is manifested by constant pain in the heart area and a pericardial friction rub. Exudative pericarditis is characterized by the accumulation of serous-fibrous exudate in the cardiac sac and is essentially the next stage of dry pericarditis. Characterized by shortness of breath, which worsens when lying down. With significant accumulation of exudate, the heart area bulges somewhat, the intercostal spaces are smoothed, and the apex beat is not palpable. The enlargement of the heart is significant; it takes the characteristic shape of a trapezoid or round carafe. The tones and noises are very dull. Often the outcome of rheumatic pericarditis is small adhesions between the outer leaf and surrounding tissues. Much less common is complete fusion of the leaves of the cardiac sac, i.e., adhesive obliterating pericarditis, the so-called armored heart, develops.

Rheumatic vascular disease. In rheumatism, the vessels in the internal organs are mainly affected (arteritis of the internal organs), which is the basis for the manifestations of rare rheumatic visceritis: nephritis, meningitis, encephalitis, etc.

Joint damage. Currently, acute rheumatic arthritis is relatively rare. Characteristic manifestations of rheumatic arthritis are increasing acute pain in the joints, aggravated by movements and palpation. Within a few hours the pain becomes extremely sharp. Very quickly, the pain is accompanied by symptoms of joint damage: swelling, sometimes hyperemia. Characterized by symmetrical damage to large joints and volatility of arthritis. Rheumatoid arthritis is completely reversible: all articular manifestations (regardless of their severity at the onset of the disease) disappear without a trace.

Currently, much more often there are only severe arthralgia without swelling of the joints, swelling is mild or completely absent, and inflammation of small joints is predominantly observed. There is also often no symmetry of the lesion. Rheumatic myositis with characteristic severe muscle pain is very rarely observed.

Skin lesions. In rheumatism, skin lesions occur in the form of rheumatic nodules, ring or erythema nodosum, urticaria, etc. Rheumatic nodules are usually located in the area of ​​the affected joints, over bone protrusions, in the occipital region, on the forearms and legs.

It is typical that under the influence of treatment (and sometimes without it) they disappear within a few days and are now practically non-existent. A very characteristic sign of rheumatic skin lesions is ring erythema, which is pink ring-shaped elements, never itchy, located mainly on the skin of the inner surface of the arms and legs, abdomen, neck and torso. This sign, like rheumatic nodules, is pathognomonic for rheumatism, but is found infrequently, in only 1–2% of patients.

Rheumatic lung lesions. Rheumatic pneumonia and pleurisy occur, but this is extremely rare. They usually occur against the background of already developed rheumatism. Distinctive features of rheumatic pneumonia are their resistance to antibiotics and the good effect of using antirheumatic drugs (without antibacterial ones). Pleurisy in rheumatism is often bilateral and easily reversible. Rheumatic nephritis is rare, and antirheumatic drugs are especially effective in their treatment.

Rheumatic lesions of the digestive organs. Such rheumatic lesions do not have significant clinical significance. Gastritis or ulcers of the stomach and intestines are consequences of long-term use of medications, especially steroid hormones. Only children suffering from rheumatism sometimes have severe abdominal pain associated with allergic peritonitis, which quickly passes, i.e., is completely reversible. The distinctive features of rheumatic peritonitis are the diffuse nature of the pain, its combination with other signs of rheumatism and the very rapid effect of the use of antirheumatic drugs. Often the pain can disappear without treatment.

In some patients with high activity of the rheumatic process, the liver may be enlarged and slightly painful due to interstitial hepatitis (damage to the connective tissue elements of the liver parenchyma).

Nervous system changes. Such changes are specific. The so-called chorea minor is a nervous form of rheumatism. It occurs mainly in children, more often in girls.

Clinically manifested by emotional instability, muscle weakness and violent, pretentious movements of the torso, limbs and facial muscles. With excitement, these movements intensify, and disappear during sleep. Minor chorea can recur, but by the age of 17–18 it almost always ends. With this form of rheumatic damage, the heart suffers slightly, and laboratory indicators of the activity of rheumatism are also slightly expressed (ESR is often not accelerated).

The central nervous system is very rarely affected by rheumatism. If this happens, the lesions usually occur as a combination of encephalitis and meningitis. Lesions of the central nervous system respond well to antirheumatic therapy.

Laboratory data. In patients with the maximum degree of process activity, there is neutrophilic leukocytosis up to 12–15? 10 3. In this case, there is a shift in the formula to the left due to the increase in band leukocytes. Metamyelocytes and myelocytes may appear in the leukogram. In most patients, the number of leukocytes and the leukogram are not significant. IN acute period During illness, the platelet count may be elevated, but this increase does not last long. Most patients with rheumatism have an accelerated ESR, reaching maximum numbers (40–60 mm/h) with polyarthritis and polyserositis. Shifts in immunological parameters are very characteristic. These include an increase in titers of antistreptococcal antibodies (antistreptohyaluronidase, antistreptokinase, antistreptolysin). An increase in the level of these antibodies reflects the body's response to exposure to streptococci, and therefore often occurs after any streptococcal infections (as does the detection of streptococcal antigens in the blood or urine). But the height of titers of antistreptococcal antibodies and their dynamics do not reflect the degree of activity of rheumatism. Many patients with chronic forms of rheumatism show no signs of streptococcal infection at all. Biochemical indicators of the activity of the rheumatic process are nonspecific, i.e. they occur when various types inflammation and tissue breakdown. In cases where the diagnosis of rheumatism is justified by clinical and instrumental data, biochemical studies are important for determining the activity of the disease.

These biochemical studies include an increase in fibrinogen levels, an increase in? 2-globulins, ?-globulins, hexoses, ceruloplasmin, seromucoid, diphenylamine reactions, etc. But the most revealing and accessible of all biochemical studies is the detection of C-reactive protein in the blood. In most cases, biochemical indicators of activity are parallel to the values ​​of ESR, which is the best laboratory sign of the activity of rheumatism, as well as its dynamics.

There are two phases of rheumatism: inactive and active. Disease activity can be of three degrees: the first degree is minimal, the second degree is average, the third degree is maximum. The activity of rheumatism is judged by the severity of clinical manifestations and changes in laboratory parameters.

IN modern conditions the nature of the disease has changed significantly. The number of patients with bright, violent manifestations and a protracted and continuously relapsing course has sharply decreased. Other visceral lesions became casuistry.

Suspicion of rheumatism should be caused by any disease that occurs 1–3 weeks after a sore throat or other nasopharyngeal infection and is characterized by signs of damage to the joints and heart. Essential diagnostic criteria are objective signs of cardiac damage, rapidly reversible arthritis of large joints, minor chorea, annular erythema and subcutaneous nodules with rapid regression. The prognosis for rheumatic lesions is based mainly on the degree of reversibility of the symptoms of rheumatic carditis. The most unfavorable are continuously recurrent rheumatic carditis, which leads to the formation of heart defects and myocardiosclerosis. Rheumatism is more severe in children. In them it more often leads to lasting changes from the heart valves. Also, the likelihood of developing heart defects increases with late treatment. If the disease primarily occurs in a patient over 25 years of age, the process, as a rule, proceeds favorably, and heart disease is extremely rare.

Reiter's syndrome, or urethro-oculosynovial syndrome

It is a disease of unknown etiology with a characteristic combination of arthritis, urethritis, conjunctivitis, and in some cases, a kind of dermatitis. The genetic characteristics of the immune system are considered likely to play a decisive role in the development of the disease. The disease mainly affects young men. The disease is often preceded by nongonococcal urethritis or acute intestinal disorder.

Clinically, arthritis varies from moderate, transient to severe, prolonged or recurrent. Most often one large joint is affected. The duration of arthritis in Reiter's syndrome ranges from 2 to 6 months, rarely longer. Many patients have spinal lesions. The severity of urethritis can vary; it is often detected only during special examinations or urine tests, i.e., it is practically asymptomatic. Conjunctivitis is usually also mild and passes quickly. In some cases there may be dermatitis. Rarely, but damage to internal organs may occur: arthritis with the development of aortic valve insufficiency, myocarditis, pericarditis, enteritis, polyneuritis, meningoencephalitis.

Laboratory findings are nonspecific. Disease activity is determined by the ESR value (acceleration) and an increase in the level of biochemical indicators of inflammation (fibrinogen, C-reactive protein, etc.). The course of the disease varies; spontaneous recovery is quite common. Making a diagnosis in the presence of the entire triad of symptoms does not cause difficulties.

Systemic scleroderma

Chronic systemic connective tissue-vascular disease characterized by progressive fibrosis. The etiology is probably viral, since when examining the affected tissues using an electron microscope, virus-like particles were detected and an increase in the titers of a number of antiviral antibodies was noted.

The pathogenetic mechanisms are quite complex and are associated with metabolic and structural disorders in collagen formation and the basic substance of connective tissue. Also in the pathogenesis, disturbances of microcirculation, as well as humoral and cellular immunity, play an important role. The role of family genetic predisposition is significant. Women get sick three times more often than men.

The onset of the disease is usually gradual, less often acute. Provoking factors are cooling, trauma, infections, vaccinations, etc. More often, the disease begins with Raynaud's syndrome (vasomotor disturbances). Also observed are tissue trophic disorders, joint pain, weight loss, asthenia, and increased body temperature. As a rule, systemic scleroderma, starting with one symptom, gradually or quite quickly becomes a generalized multisyndromic disease.

The pathognomonic (specific) sign of the disease is skin lesions. This is a common dense swelling, and later – thickening and atrophy of the skin. The greatest changes occur in the skin of the face and limbs. But often the skin of the entire body becomes dense. At the same time, focal or widespread pigmentation develops with areas of depigmentation and dilation of small vessels. Characteristic are ulcerations and pustules on the fingertips, which are very painful and do not heal for a long time, nail deformation, hair loss (even baldness) and other trophic disorders.

Fibrolyzing interstitial myositis is often observed. Muscle syndrome manifests itself as muscle pain, progressive hardening, then muscle atrophy, and decreased muscle strength. In rare cases, many muscles are affected (acute polymyositis) with pain, muscle swelling, etc. The replacement of muscle fibers with connective tissue is also accompanied by tendon fibrosis, which leads to muscle-tendon contractures, which are one of the main causes of early disability in patients. In 80–90% of cases, joint pain is observed, often accompanied by joint deformation, often quite pronounced due to changes in the periarticular tissues.

X-rays do not reveal significant destruction. Important diagnostic sign is osteolysis (resorption) of the terminal, and in severe cases, the middle phalanges of the fingers, and less often of the toes. With scleroderma, deposits of calcium salts are observed in the subcutaneous tissue. These deposits are localized mainly in the area of ​​the fingers and in the periarticular tissues and appear in the form of uneven, painful formations that can spontaneously open with the rejection of crumbly calcareous masses.

Almost all patients are affected by the cardiovascular system such as myocarditis, endocarditis, and rarely pericarditis. As a result of inflammatory lesions of the heart, sclerodermic cardiosclerosis is formed, which is clinically manifested by pain in the heart area, shortness of breath, arrhythmia in the form of extrasystole, muffled tones, systolic murmur at the apex, and expansion of the heart to the left. Localization of the process in the endocardium leads to the formation of scleroderma heart disease. The mitral valve is usually affected. Scleroderma heart disease is characterized by a benign course. Heart failure rarely develops, only with widespread, pronounced myocarditis or with damage to all the membranes of the heart at once.

Peripheral symptoms of scleroderma are caused by damage to small arteries and arterioles. The consequences of these lesions are Raynaud's syndrome, telangiectasia, and gangrene of the fingers. Damage to the blood vessels of internal organs leads to severe visceral pathology. Hemorrhages, ischemic phenomena and even necrotic changes in organs are observed. There may be decay of lung tissue, true scleroderma kidney, etc. Vascular pathology determines the speed of the process, its severity and even the outcome of the disease. It is also possible to damage large vessels with a picture of obliterating thromboangiitis, the development of ischemic phenomena, migrating thrombophlebitis with trophic ulcers in the area of ​​​​the feet and legs, etc. Damage to the lungs is usually accompanied by emphysema and bronchiectasis due to focal or diffuse pneumofibrosis. Focal nephritis most often develops in the kidneys, but in some cases diffuse glomerulonephritis with hypertensive syndrome and renal failure is possible.

Damage to the nervous system is manifested by polyneuritis, autonomic instability, characterized by impaired sweating, thermoregulation, and vasomotor reactions of the skin. There may also be emotional lability, irritability, tearfulness, suspiciousness, insomnia. In very rare cases, a picture of encephalitis or psychosis occurs. Due to scleroderma damage to the blood vessels of the brain, symptoms of sclerosis are possible even in young people. Possible lesions of the reticuloendothelial system, which are manifested by an increase in multiple lymph nodes and the spleen, as well as damage to the endocrine system in the form of pathology of any endocrine gland. In the subacute course, the disease begins with joint pain, fever, weight loss, and pathology in the internal organs quickly increases. In this case, the disease takes on a steadily progressive course with the spread of the pathological process to many organs and systems. Patients usually die within 1–2 years from the onset of the disease. A chronic course is much more common. The disease lasts for decades with minimal process activity and gradual spread of lesions to internal organs, the functions of which are not impaired for a long time.

Patients suffer mainly from damage to the skin, joints and trophic disorders. In chronic systemic scleroderma, calcification, Raynaud's syndrome, telangiectasia, and finger lesions are distinguished. All these pathologies are characterized by a long-term benign course with extremely slow development of damage to internal organs. Laboratory findings are not typical. Usually there is moderate leukocytosis and eosinophilia, transient thrombocytopenia. ESR is normal or moderately accelerated in chronic cases and very high (up to 50–60 mm/h) in subacute cases.

Ankylosing spondyloarthritis (Bechterew's disease)

Chronic inflammatory disease of the joints of the spine with a tendency to develop gradual limitation of movements in them. The etiology and pathogenesis are not yet clear. Great importance is attached to the genetic characteristics of the immune system. The disease mainly affects men.

An obligatory symptom of ankylosing spondylitis is damage to the spine. But this lesion is often limited for a long time only to the sacroiliac joints (sacropleitis). Manifestations of sacropleitis can be vague (in the form of discomfort, mild pain) and inconsistent. Sometimes subjective sensations may be completely absent, and only x-ray examination reveals damage to the sacroiliac joint. As the small joints of the spine become involved in the process, pain appears in one or another part of it (sometimes in the entire spine). Very often the pain intensifies at night, and in the morning there is stiffness. Later, restrictions on the movements of the spine are added: the patient cannot reach the floor with his fingers without bending his knees, or the sternum with his chin; there is a decrease in the respiratory excursion of the chest. The physiological curves of the spine gradually smooth out, hyperkyphosis forms thoracic, i.e., a very characteristic pose of a petitioner appears. The course of this form of ankylosing spondylitis (central) is usually slow, long-term, with periods of exacerbations and remissions. Damage to non-vertebral joints is also typical, and has some peculiarities. Large joints of the lower extremities (hip, knee, ankle) are most often affected, often also the shoulder and sternoclavicular joints. Oligoarthritis and asymmetrical joint damage (peripheral form) are typical. Most often, the disease is short-lived (1–2 months), but it can also be protracted.

Muscle pain, especially in the back, and the development of inflammation in the Achilles tendon are also characteristic. In some cases, internal organs are affected: eyes (iris damage), aorta (aortitis), myocardium (sometimes with impaired atrioventricular conduction), endocardium with the formation of valve insufficiency, kidneys (glomerulonephritis, urethritis). With a long course, amyloidosis often develops, predominantly affecting the kidneys.

Diagnosis is based on x-ray examination(radiography), where characteristic changes are detected. Sacropleitis is the earliest radiological symptom of spinal damage; in some cases, it develops within 4–6 months from the onset of the disease.

Sjögren's syndrome

It is a chronic inflammation of the endocrine glands, mainly salivary and lacrimal, leading to their secretory insufficiency. May be isolated syndrome(this is the so-called dry syndrome). The name speaks for itself, since the most striking clinical signs are dry mouth and eyes. The cause of the disease has not been fully elucidated, but the most likely opinion is that it is of autoimmune origin, which is confirmed by the frequent combination with other diseases of an autoimmune nature: rheumatoid arthritis, systemic lupus erythematosus, systemic scleroderma, etc. Mostly middle-aged women are affected. Sjogren's syndrome is characterized by a combination of dry keratoconjunctivitis (xerophthalmia) and dry stomatitis (xerostomia), which are associated with damage to the lacrimal and salivary glands and secretory insufficiency. There are also recurring parotitis (lesions parotid glands), usually symmetrical, pain and swelling in the area of ​​the submandibular glands. Dry eyes (xerophthalmia) is manifested by a constant burning sensation, sensation of a foreign body in the eyes, photophobia, a sharp decrease or complete disappearance of tears. Consequences of persistent dry mouth include difficulty chewing and swallowing. Glossitis (inflammation of the tongue), cheilitis (inflammation of the red border of the lips), and progressive dental caries develop.

Patients are bothered by constant pain in the joints and occasional swelling, but there is no severe deformation or destruction with sicca syndrome. Raynaud's syndrome is also observed, and drug intolerance is common. Laboratory data are quite characteristic: rheumatoid factor is positive, ESR is accelerated. Diagnosis is based on two of three features: xerophthalmia, xerostomia and autoimmune disease. Sjögren's syndrome occurs as a chronic relapsing disease involving the lymph nodes and internal organs.

In addition to such a large group of acquired immunodeficiencies as collagenosis, which occur as autoimmune lesions, there are also autoimmune diseases of other body systems. For example, these include diseases of the blood system (agranulocytosis, autoimmune hemolytic anemia), nervous system (multiple sclerosis).

Agranulocytosis

Agranulocytosis is a decrease in the number of leukocytes (less than 1000 in 1 μl of blood) or the number of granulocytes (less than 750 in 1 μl of blood). As a rule, agranulocytosis is a symptom of some general disease. The most common are myelotoxic agranulocytosis (cytostatic disease) and immune agranulocytosis. Immune agranulocytosis is caused by the appearance of autoantibodies (for example, in systemic lupus erythematosus) and antibodies to granulocytes after taking medications (so-called haptens). Haptens are medications that, when they enter the body, combine with a protein and acquire the properties of an antigen. Hapten agranulocytosis is caused by diamox, amidopyrine, antipyrine, acetylsalicylic acid, barbiturates, isoniazid (tubazid), meprobamate, phenacetin, butadione, plasmoquine, indomethacin, levamisole, sulfonamides, biseptol, chloroquine, antidiabetic sulfonamide drugs, insecticides (drugs for insects).

The mechanism of development of agranulocytosis has not been sufficiently studied. At autoimmune forms lesions, premature death of granulocytes and their bone marrow precursors is caused by autoantibodies. The mechanism of the individual reaction of the body to the ingestion of a drug during hapten agranulocytosis is not yet clear. It is characteristic that, once occurring, hapten agranulocytosis will invariably recur when the same drug, hapten, is introduced into the body. Clinical manifestations are caused by agranulocytosis itself (i.e., a sharp decrease in the number of leukocytes, protective cells). Therefore, septic complications are typical: tonsillitis, pneumonia, etc. Laboratory tests do not detect granulocytes in the blood, but the number of lymphocytes, platelets, and reticulocytes is normal. There is no bleeding or hemorrhage. Occasionally, antibodies to platelets may also appear, and thrombocytopenic hemorrhagic purpura occurs. The prognosis of autoimmune agranulocytosis is determined by the underlying diseases (systemic lupus erythematosus, rheumatoid arthritis, etc.). Hapten agranulocytosis gives a high percentage deaths(up to 80%). The prognosis is sharply aggravated by repeated exposure of haptens to the body. Since it is often very difficult to establish which particular drug was the hapten, it is necessary to exclude all suspected medications from use for life. This rule is the main preventive measure for repeated hapten-type agranulocytosis.

Immune hemolytic anemias

These are anemias that are caused by the effect of antibodies on red blood cells. There are several forms of immune hemolytic anemias. These are autoimmune anemias caused by the formation of antibodies in the body against its own red blood cells; hapten, caused by fixation on erythrocytes of hapten antigens foreign to the body (drugs, viruses, etc.) with antibodies formed in response to the combination of a hapten with a protein of the body; isoimmune, associated with the entry into the body of the newborn of maternal antibodies directed against the child’s red blood cells (in case of incompatibility of the child and mother with respect to the Rh factor and much less often with respect to blood group).

Autoimmune hemolytic anemias

The pathological process is based on a breakdown of immunological insensitivity to one’s own antigen. The leading sign of the clinical picture is anemic syndrome. The severity of the condition is determined by the severity and severity of anemia. When the process develops slowly, the first sign of the disease may be slight jaundice (due to indirect bilirubin), and anemia is also detected at the same time. In other cases, the onset of the disease is rapid, with hemolysis (decomposition of red blood cells), rapidly increasing anemia, and jaundice. Body temperature often rises. Sometimes the spleen and liver become enlarged. A systolic murmur is heard at the apex and base of the heart, which is of a functional nature. A blood test reveals normochromic anemia, and in the acute course of the disease, the hemoglobin level can drop to catastrophic levels. Then the patient may fall into an anemic coma. In acute hemolysis, single erythrokaryocytes can be detected in the blood. The level of reticulocytes is also high. The leukogram does not change significantly, but a hemolytic crisis may be accompanied by short-term neutrophilic leukocytosis. The platelet count is usually normal. However, autoimmune cytolysis (cell breakdown) occurs, affecting two germs: platelet and erythrocyte (Evens-Fisher syndrome). In this case, there are signs of hemolytic anemia and thrombocytopenic purpura. With autoimmune hemolytic anemia, irritation of the red sprout occurs in the bone marrow, i.e., when hemolysis is combined with thrombocytopenia, high megakaryocytosis is observed in the bone marrow. In a biochemical study, in addition to hyperbilirubinemia, an increase in β-globulins is noted.

It is impossible to give a prognosis for the disease. It may be a single episode of red blood cell breakdown, or it may develop into a chronic hemolytic process. In addition to this, the most common form of autoimmune hemolytic anemia, in which hemolysis occurs intracellularly, there is a form of the disease with intravascular hemolysis. The difference between these forms is that with intravascular hemolysis, dark urine is released due to hemoglobinuria and hemosiderinuria. With severe hemolysis, thrombosis in the mesenteric vascular system is possible with the appearance of severe paroxysmal pain in the abdominal area. In rare cases, intravascular hemolysis may occur during cooling (cold hemoglobinuria). Another form of autoimmune hemolysis is associated with exposure to cold, in which intracellular hemolysis occurs, provoked by cooling the body. In this case, autoagglutination (gluing) of red blood cells is noted immediately after taking blood from a finger when it is cooled to room temperature.

The diagnosis of autoimmune hemolytic anemia is made on the basis of general signs of hemolysis: an increase in the level of bilirubin in the blood or the appearance of bilirubin in the urine, an increase in the percentage of reticulocytes in the blood and the detection of autoantibodies on the surface of red blood cells using the Coombs test (a special laboratory test), which is positive in almost 60% cases of autoimmune hemolysis.

Multiple sclerosis

A disease of the nervous system, which is based on the occurrence of scattered brain and spinal cord foci of demyelination, which either disappear over time or are replaced by plaques (glial scars). The cause of this disease is not clear enough. Most likely, the mechanism involves autoimmune reactions. The demyelinating process primarily affects the white matter of the central nervous system. The affected area undergoes remyelination; after the breakdown of myelin, the axial cylinders are also damaged, followed by the formation of a characteristic dense glial plaque ranging in size from several millimeters to several centimeters. Remyelination (restoration of myelin) underlies clinical remissions. With the development of scars, the functions of the affected areas of the central nervous system are lost irreversibly.

The disease usually occurs at a young age. In childhood and after 50 years, the disease develops extremely rarely. The first symptoms of the disease are transient motor, sensory (usually numbness) or visual disturbances. Over time, newly emerging lesions are no longer subject to reverse development. There is a steady increase in the severity of the clinical picture. The pyramidal and cerebellar systems and optic nerves are most often affected. Almost always (in 90% of cases) in the advanced stage of the disease there is lower spastic paraparesis or tetraparesis (weakness in the lower extremities or in the upper and lower extremities). At the same time, cerebellar disorders are expressed: gait disturbances, speech disturbances, involuntary movements of the eyeballs (nystagmus). A pronounced tremor of the limbs and head is observed, and trembling is detected during active movements and tension, but can also be at rest. The combination of nystagmus, speech disturbances (chanted speech) and tremors together form Charcot's triad, which is a characteristic feature of multiple sclerosis.

Damage to the optic nerves leads to decreased visual acuity. In the fundus there is blanching of the temporal discs. Urinary disturbances are common. Many patients have a kind of euphoria, and in advanced cases, dementia (dementia) is common. In approximately 85% of cases, multiple sclerosis is characterized by a remitting course, that is, periods of exacerbation are replaced by significant improvement, and often the complete disappearance of all or individual signs of the disease. The duration of improvements can range from several hours to several years. Particularly good remissions are observed in the first years of the disease. However, after a few years, most patients become disabled to one degree or another. In advanced and irreversible stages of the disease, the combination of paresis with ataxia (staggering gait) is especially characteristic. The onset of the disease in many patients may be preceded by febrile illnesses, vaccinations, injuries, surgeries, and pregnancy.

The diagnosis is confirmed by studies of the cerebrospinal fluid, in which in almost 90% of cases there are certain anomalies, for example, a moderate increase in protein, a paralytic type of Lange colloid reaction, and an increase in the level of β-globulins.

AIDS

AIDS is an acquired immunodeficiency syndrome that is caused by the human immunodeficiency virus (HIV), so the disease has two names: AIDS or HIV infection. The human immunodeficiency virus was isolated in 1983 by French and then American researchers. The detection of the virus in certain substrates associated with sick people (blood, saliva, semen) made it possible to clarify the routes of transmission of the disease. In turn, the establishment of the etiology made it possible to develop work on the serological diagnosis of the infection. Thus, AIDS was clearly differentiated from other acquired immunodeficiencies.

AIDS is a severe disease; with advanced disease, the death of the patient is almost inevitable. In terms of mortality, AIDS has taken third place after atherosclerosis and cancer. True, this applies to forms of the disease with a pronounced clinical picture. Despite the fact that AIDS cannot be called a widespread disease, the increase in the number of cases, according to scientists, is growing in geometric progression. It is believed that the number of cases doubles every six months. It is also alarming that, according to the latest data, the population that has antibodies to the virus that causes AIDS amounts to millions. All this raises concerns that acquired immunodeficiency may become a widespread disease in the future. There is also a wide geographical spread of AIDS. Currently, there is not a single inhabited continent free from this disease.

The human immunodeficiency virus is a so-called retrovirus. Retroviruses are the only living creatures in the world that can synthesize DNA with RNA, while others can only synthesize RNA with DNA. For this purpose, viruses of this group have the enzyme reverse transcriptase. Hence the name retrovirus (from the Latin “retro” - “reverse”). Among animal viruses that cause immunodeficiency states, monkey retroviruses are of greatest interest. Once in the human body, the human immunodeficiency virus attaches to special formations located on the lymphocyte cell, then penetrates inside it, integrates into the genetic apparatus of the cell and forces the production of virus particles until the cell dies. New viruses infect new cells, etc. It can take a dozen years before the number of lymphocytes decreases to such an extent that immunodeficiency develops. But all this time, an infected person, feeling healthy, can be a source of infection for others.

This infection has a number of clinical and epidemiological features. These include:

1) an unusually (for the vast majority of infections) long incubation period (sometimes exceeding 5 years), so AIDS can be classified as a so-called slow viral infection;

2) an extremely “narrow” application of the virus - it affects only some categories of immunocompetent cells, but this does not prevent the occurrence of a total defeat of the entire defense system of the body;

3) the infection does not have a specific clinical picture - its manifestations are determined by opportunistic states (i.e., adapting to certain conditions), the clinical picture of which is extremely diverse, which makes a purely clinical diagnosis of the disease impossible.

Many features of the disease currently defy rational explanation. Remains unclear origin AIDS. However, the mechanism of action of the AIDS virus on the body has already been sufficiently studied and the clinical manifestations of the disease in its advanced stage have been described. The main thing in the pathogenesis of HIV infection is the identified ability of the virus to selectively turn off T-helper cells, as a result of which the immune response does not develop, and the person becomes completely defenseless against any infection or pathology (can even die from opportunistic bacteria). The virus, entering T-helper cells, can remain in an inactive state for many years, but the person is already infected. When HIV becomes active for some reason, AIDS develops, and most patients die within 1–2 years.

Pathoanatomical changes in those who died from AIDS are diverse and largely depend on the nature of the opportunistic diseases that led to death. In those who died from AIDS, common inflammatory and suppurative processes are found: lung abscesses, damage to the liver, kidneys, heart, and lymph nodes. Ulcerations of the esophagus and intestines were noted. If there have been infections (toxoplasmosis and cryptococcosis), then corresponding changes are detected in the brain substance.

Histological examination of the material shows the absence of granulomas as a characteristic sign of AIDS. At electron microscopy in biopsy samples of various tissues, multiple tubular-reticular inclusions in the cytoplasmic reticulum of endothelial cells, histocytes and lymphocytes are revealed. In preparations made from bronchial swabs, saliva, urine, and gastric juice, pronounced cellular atypia and an increase in mature and immature lymphoreticular elements are found. In the bone marrow, a normal and slightly increased number of nuclear cells with a normal ratio of myeloid and erythrocyte cells, moderate plasmacytosis and a slight increase in reticulin are noted. The number of lymphocytes is reduced. The bone marrow aspirate contains histiocytes, many of which are engulfed by nucleated erythroid cells or granulocytes, which is similar to the virus-associated phagocytic syndrome described in patients with immune system dysfunction. In the lymph nodes there is intense follicular hyperplasia, the size and shape of the follicles, disturbances in cellular composition, similar to those found in the blood, in particular the predominance of T-suppressors. The pathology of the thymus in children with AIDS has been studied. A sharp decrease in the number of lymphocytes and Hassal bodies was noted. In those who died from the malignant course of AIDS, there was no division into the cortical and medulla layers in the thymus gland, and Hassall's bodies and accumulations of epithelial cells were not detected. The thymus tissue was infiltrated with plasma cells and mast cells.

Changes in the thymus in AIDS and congenital immunodeficiency are associated with damage to the T-system, but careful pathological and anatomical study makes it possible to clearly differentiate AIDS from congenital immunodeficiency.

AIDS is characterized by a normal anatomical position and configuration of the thymus with normal blood vessels. The described changes in immunodeficiency and one of the central organs of the immune system (thymus gland) lead to serious disturbances in its function. Delayed hypersensitivity reactions (to tuberculin, streptokinase, trichophytin) are sharply suppressed. The proliferative activity of lymphocytes when stimulated by soluble antigens is reduced. At the same time, the level of immunoglobulins (JgM, JgJ, JgA) is increased.

The presence of lymphocytotoxic antibodies in the blood serum of AIDS patients, which are combined with a deficiency of cellular immunity, has been established. AIDS patients lack the synthesis of interleukin-2. The production of interleukin-2 is inhibited by hypersecretion of prostaglandins. After isolating the causative agent of AIDS and developing methods for determining antibodies to the virus, it was found that the number of people with antibodies to the causative agent significantly (about 50-100 times) exceeds the number of patients with clinically manifest AIDS. As for the routes of transmission, there is no doubt that AIDS is transmitted through direct contact during sexual intercourse. Another route of transmission of infection is through household contact - through objects contaminated with the blood of sources of infection, when the virus enters the body through minor defects in the skin and mucous membranes. There is no doubt about the possibility of “vertical” transmission of infection from virus-carrying mothers or patients. Already the first works of US scientists made it possible to identify a population that has an increased risk of developing AIDS, i.e., the so-called risk groups. This includes homosexuals, drug addicts who inject drugs intravenously, patients with hemophilia, and people receiving numerous blood transfusions.

Characterizing the clinical picture of this serious and dangerous disease, there is reason to distinguish three main forms of infection: asymptomatic; an infection that occurs as a generalized lymphadenopathy and AIDS itself, when, in addition to the general symptoms characteristic of immunodeficiency, various opportunistic diseases occur with a predominant lesion of certain systems. The main feature of this infection is the length of the incubation period. Without a doubt, AIDS is an infection with a very long incubation period (from several months to several years). Moreover, the duration of incubation for different age groups not the same. For example, in patients who had homosexual contact with other AIDS patients, the incubation period ranged from 9 to 22 months. With blood transfusion, incubation can last up to 58 months. The average duration of the incubation period in children is 12 months, in adults – 29 months; if infected through a blood transfusion, incubation increases by 4 years.

At the end of the incubation period, the disease phase begins, which in various sources is designated by different terms: generalized lymphadenopathy, persistent generalized lymphadenopathy, side AJDS complex, lymphadenopathy syndrome, chronic lymphadenopathy, prolonged unmotivated lymphadenopathy syndrome, prodrome phase, pre-AIDS. It is believed that generalized lymphadenopathy in some cases is a transitional phase of the development of infection (prodrome, pre-AIDS), in other (favorably current) cases, the clinical picture of the disease does not develop further, i.e. generalized lymphadenopathy ends with recovery and acts as an independent form of the disease.

All of the above names for this condition emphasize a characteristic feature - lymphadenopathy. In patients, lymph nodes enlarge in several areas of the body at once. Diagnostic value has an enlargement of at least two groups of lymph nodes outside the groin area. Lymph nodes are moderately painful (but can also be painless), not associated with fiber, mobile, 1–3 cm in diameter. The duration of lymphadenopathy is very characteristic - at least 3 months, often over a number of years. In addition to lymphadenopathy, this condition causes a recurrent temperature reaction, night sweats, and increased fatigue. Characteristic signs are weight loss (decrease in body weight by at least 10%), as well as chronic diarrhea. Skin manifestations are less common: rashes, in some cases fungal diseases, seborrheic dermatitis of the face, frontal baldness.

Laboratory testing reveals lymphopenia, a change in the ratio of T-helpers to T-suppressors in favor of T-suppressors, a decrease in the response of T-cells to mitogens, and impaired delayed-type hypersensitivity reactions. The presence of antibodies to the human immunodeficiency virus (HIV) is detected in approximately 80% of patients. The levels of JgM, JgJ and JgA are increased. Increased amount of LJ-thymosin. Thus, laboratory data correspond to classic AIDS, but are less pronounced. In a minority of patients with generalized lymphadenopathy (about 1 in 10), the disease develops into “real” AIDS.

Characterizing the course of AIDS, researchers point out that the AIDS clinic does not have clear nosological contours. A peculiar combination of causal and co-causal factors arises, creating a severe pathology, the nature of which is determined by opportunistic infections.

Early signs of AIDS are aggravated symptoms of the previous period - the pre-AIDS period:

1) fever of unknown etiology with a course that is not amenable to conventional treatment;

2) lymphadenopathy;

3) increasing general weakness;

4) loss of appetite;

5) diarrhea;

6) weight loss;

7) enlargement of the liver and spleen;

8) cough;

9) leukopenia with the possible addition of erythroblastopenia.

Later, visual disturbances associated with retinitis (inflammation of the retina of the eyes) may occur. There are several types of disease. Respiratory system lesions are the most common manifestation of AIDS. They were noted in 60% of patients. The so-called pulmonary type includes hypoxemia, chest pain, and diffuse pulmonary infiltrates on radiography. The most common opportunistic infection associated with lung damage is Pneumocystis pneumonia, while Legionella lung disease and cytomegaly are much less common.

Lesions of the central nervous system are observed in approximately 1/3 of AIDS patients, and several main forms are distinguished:

1) abscesses caused by toxoplasma;

2) progressive multifocal leukoencephalopathy;

3) cryptococcal meningitis, subacute encephalitis (usually cytomegalovirus etiology);

4) tumors, such as primary and secondary brain lymphomas;

5) vascular lesions (non-bacterial thrombotic endocarditis and cerebral hemorrhage associated with thrombocytopenia);

6) lesions of the central nervous system with focal brain damage with non-spread (self-limiting) meningitis.

In addition to infection, hypoxic phenomena and thromboembolism have been observed in patients with AIDS. According to clinical observations, in approximately 25% of patients the immediate cause of death was damage to the central nervous system. As a result of clinical studies, data were obtained about the possibility of indefinitely long-term persistence of the AIDS virus in brain cells, from where the pathogen can enter the blood, causing disorders of the immune system. The AIDS virus located in brain cells can cause dementia (dementia), which is not associated with damage to the immune system.

In patients with AIDS, the kidneys are affected, and glomerulonephritis with nephrotic syndrome is more common. Most patients with kidney pathology in AIDS quickly develop end-stage renal failure. A pathological examination reveals focal segmental glomerulonephritis with deposition of JgM in the glomeruli. Approximately 40% of AIDS patients experience various ophthalmological lesions: conjunctivitis, keratitis, retinitis, retinal periphlebitis, retinal hemorrhages, the appearance of a white spot, which causes decreased vision. It is characteristic that the appearance of a white spot and cytomegalovirus retinitis is a negative prognostic sign. Skin lesions most often manifest as Kaposi's sarcoma, but are not limited to it. Seborrheic dermatitis, folliculitis, vasculitis, xerodermatitis, herpes zoster, and various manifestations of fungal infection may also occur.

The most common opportunistic conditions encountered in AIDS are grouped according to etiology as follows:

1) malignant neoplasms: Kaposi's sarcoma, brain lymphoma;

2) invasions: Pneumocystis pneumonia, toxoplasmosis, causing pneumonia or damage to the central nervous system, cryptosporidiosis (intestinal form with prolonged diarrhea), stronglioidosis (pneumonia, damage to the central nervous system, disseminated process);

3) mycoses: candidiasis (most often of the esophagus and oral cavity), cryptococcosis (damage to the lungs, central nervous system, disseminated process);

4) bacterial infections: pneumonia caused by Legionella, atypical mycobacteriosis (disseminated infection), salmonella infection (enteritis, sepsis);

5) viral infections: cytomegalovirus infection (damage to the lungs, gastrointestinal tract, central nervous system), progressive leukoencephalopathy (apparently caused by papavirus), infections caused by herpes viruses, infections caused by HTLV-I and HTLV-II viruses. But with all the diversity of opportunistic conditions, a number of the most common ones can be identified. These are, firstly, Pneumocystis pneumonia and Kaposi's sarcoma. According to numerous sources, approximately 50% of AIDS patients have Pneumocystis pneumonia as an opportunistic disease, and 25% have Kaposi's sarcoma. About 6% of patients are affected by both conditions. Less than 20% of opportunistic diseases are caused by all other infectious agents, with the most common infections being caused by cytomegalovirus, herpes virus and Candida fungi.

Pneumocystis pneumonia

The causative agent of the disease is pneumocystis, a type of protozoan, first described in 1909. This microorganism can cause interstitial pneumonia in premature and weakened children. The disease has a wide geographical distribution, but is quite rare. The disease occurs extremely rarely in adults suffering from blood diseases, tumors, in persons treated with corticosteroids and immunosuppressants, and during organ transplantation. There are known cases of generalized infection. In Pneumocystis pneumonia, inflammatory infiltration of the interalveolar septa leads to the filling of the alveoli with a foamy mass, which reduces the respiratory surface of the lungs, causing impaired gas exchange and oxygen deficiency.

Clinically, the disease develops gradually; in some cases there may be a wave-like current. At the beginning, rapid breathing, shortness of breath, and cyanosis appear. The temperature is often subfebrile. Subsequently, shortness of breath, rapid breathing, and cyanosis progress, which are later joined by a dry, obsessive cough, respiratory acidosis, and the possible formation of pneumothorax. Pulmonary heart failure develops. The liver and spleen enlarge. Pneumocystis pneumonia can be complicated by a bacterial infection.

A presumptive diagnosis can be made on the basis of clinical, epidemiological data and a characteristic x-ray picture, the final diagnosis can be made on the basis of detection of the pathogen in the mucus of the upper respiratory tract, as well as using an immunofluorescence reaction. This infection only affects people; it spreads through airborne droplets and dust. Pneumocystis pneumonia in patients with AIDS often recurs and has an exclusively malignant course with a mortality rate of 90 to 100%, whereas usually this disease is relatively mild.

Kaposi's sarcoma

First described in 1872. Also known under many other names (about 70 terms). Kaposi's sarcoma is a malignant tumor disease of the reticulohistiocytic system with a predominant involvement of the skin. According to the classification of skin tumors, Kaposi's sarcoma belongs to malignant diseases of blood vessels - hemorrhagic hemangioendotheliomas.

Clinically, during the normal course of the disease (not in patients with AIDS), skin lesions appear in the form of spots, plaques, nodes with areas of hemorrhage. The lesions are characterized by symmetry. The size of the elements is up to 5 cm in diameter, the color is reddish-bluish, reddish-brown, later the color becomes darker. The elements are sharply limited from the surrounding skin, their surface is smooth with slight peeling. No pain is felt. There is a gradual increase in the elements in size and number, their grouping in the form of arcs and rings with subsequent compaction, retraction of the center, formation of plaques and tumor nodes 1–5 cm in size, hemispherical in shape, protruding above the surface of the skin. Ulceration of tumors is possible. Kaposi's sarcoma is most often localized on the anterior surface of the leg, much less often - on the ears, abdomen, and penis. Sometimes elephantiasis of the extremities develops (severe swelling due to stagnation of lymph), sharp pain in tumor-like formations appears, and generalization of the process is noted with the formation of tumor nodes in the gastrointestinal tract, liver, lungs, lymph nodes and bones. Kaposi's sarcoma, not associated with AIDS (as an independent disease), in 3/4 of cases has a long (6-10 years, less often - 15-20 years) course. Less commonly, a subacute course is observed (2–3 years); in some cases - an acute form with rapid death of patients. Without an association with AIDS, Kaposi's sarcoma is a rare disease (0.06 per 100,000 population), although it has recently become significantly more active. As a rule, men over 60 years of age become ill. The highest incidence was observed in the indigenous population of Central Africa. There are European, African and North American variants of the disease. Kaposi's sarcoma, which occurs in patients with AIDS, is histologically no different from normal, but has a number of features. It does not primarily affect the lower extremities, but is associated with the lymph nodes, mucous membranes and membranes of internal organs. The disease acquires a disseminated malignant nature. There may also be a lightning current. There is an opinion that Kaposi's sarcoma is an opportunistic disease in AIDS due to the fact that the AIDS virus induces tumorigenesis by stimulating B-cell proliferation with a predominance of one clone.

Candidiasis

This is a disease caused by yeast-like fungi of the genus Candida. A clinically pronounced disease develops, as a rule, when the functions of the protective system are impaired, which is primarily characteristic of AIDS. The most common localization of candidiasis in AIDS is the oral cavity, and especially the esophagus. There may also be skin candidiasis and a common form (up to 80%).

Cytomegalovirus infection

Caused by a virus of the same name. The name of the disease is associated with the mechanism of development of the infection. In the affected tissues, giant cells with characteristic intranuclear inclusions are formed (from the Greek citos - “cell” and megalos - “big”). There may be changes in the lungs, gastrointestinal tract and central nervous system. In the pulmonary form, interstitial pneumonia occurs, and sometimes multiple cysts form in the lungs. In the gastrointestinal form, persistent diarrhea with abdominal pain occurs. Ulcerative enteritis and sometimes pancreatitis are noted. When the central nervous system is damaged, the clinical picture of meningoencephalitis develops. In the absence of AIDS, cytomegalovirus infection affects only children. In AIDS, cytomegalovirus infection is found in 70% of patients. The malignant nature of this infection is usually noted.

Infections associated with herpes viruses

Diseases caused by herpes simplex viruses and herpes zoster virus are less common in patients than diseases associated with cytomegaly virus. Of the two herpes viruses, opportunistic infections caused by the herpes simplex virus are more common. As a rule, with AIDS these diseases are malignant. Interstitial pneumonia, chorioretinitis (eye damage), hepatitis, damage to the kidneys, brain, and endocrine glands develop. Infection caused by herpes zoster is observed half as often. Herpes zoster, which occurs without connection with AIDS, most often affects people over 60 years of age. In AIDS, this infection occurs in people aged 20–30 years. Opportunistic conditions in AIDS have a number of features.

1. Opportunistic pathogenic microorganisms are often used as pathogens, which under normal conditions do not cause pathological processes or cause them only in a certain group (young children, elderly people treated with hormones or irradiated).

2. The pathogens are microorganisms that stay in the body for a long time and do not cause pathology in its normal state.

3. Opportunistic infections that complicate AIDS are characterized by a malignant course, a tendency to spread, duration, and high mortality.

4. Opportunistic infections often recur; one infection can change to another; sometimes several opportunistic diseases occur simultaneously.

All these features are due to the pathogenesis of the disease itself – a sharp suppression of the immune system.

Features of the course of AIDS in children. Children make up a relatively small proportion of AIDS patients. They are mainly infected in utero, as well as through blood transfusions and treatment of hemophilia. On average, the disease occurs 5 months after birth. Children with AIDS exhibit prolonged fever, underdevelopment, hypergammaglobulinemia, and impaired cellular immunity. Pneumocystis and cytomegalovirus pneumonias and Salmonella sepsis predominate as opportunistic infections. Some sick children simultaneously experience several forms of infections and pathologies caused by different etiological factors. Kaposi's sarcoma is very rare in children with AIDS. At the same time, infections caused by bacterial microflora are found in children more often than in adult patients. In children under one year of age, diarrhea is especially common.

Diagnosis of AIDS. Making a diagnosis of AIDS is an extremely difficult and responsible task. Overdiagnosis is completely unacceptable. The difficulty of diagnosing AIDS is primarily due to the polymorphism of the clinical picture of the disease due to the wide variety of opportunistic conditions. Many of them require rather complex laboratory diagnostics. If there is a combination of clinical data with an immunodeficiency state confirmed by appropriate tests, then the diagnosis becomes justified. But even in these cases, caution is required, since immunodeficiency states can be etiologically and pathogenetically different. It is impossible to equate AIDS with immunodeficiency, even T-cell deficiency. Specific serological tests play an important role in making a diagnosis, but they must be performed repeatedly. Only a combination of epidemiological, clinical, immunological and specific serological methods diagnostics allows specialists to diagnose AIDS. Careful collection of anamnesis and dynamic observation of the patient make it possible to identify a symptom complex characteristic of pre-AIDS: lymphadenopathy, weight loss, persistent diarrhea, febrile reaction. Each of these symptoms in itself is of little evidence, but in combination with a risk population (drug addicts, prostitutes, etc.) they allow one to suspect pre-AIDS. Since the emergence of opportunistic conditions, the grounds for diagnosing AIDS have become significantly greater. This is especially true for opportunistic conditions most characteristic of AIDS, such as Pneumocystis pneumonia, Kaposi's sarcoma, candidiasis, and cytomegalovirus infection.

Before the development of specific serological and virological tests, the diagnosis of AIDS was made on the basis of clinical data and immunological tests, subject to the exclusion of all other factors that could cause immunodeficiency (primary immunodeficiencies, immunodeficiencies caused by radiation, chemotherapy, fasting, administration of adrenal hormones - corticosteroids).

Diagnosing AIDS in children is especially difficult, since in early childhood the immune system is not yet fully formed, and opportunistic infections are possible in newborns even in the absence of AIDS. In children, when diagnosing AIDS, collecting anamnesis (disease history) is of great importance. The history concerns both the child himself (does he have hemophilia, have he had blood transfusions) and his parents (drug addiction, numerous sexual contacts, arrival from AIDS areas).

If AIDS is suspected, based on epidemiological and clinical data, it is advisable to study the state of the immune system and determine the nature of the disorders. The complexity of an immunological examination is determined by the difficulties in correctly assessing the results obtained and the technical formulation of reactions, which are not available to all laboratories. Patients with manifest (pronounced) forms of AIDS are characterized by changes in the form of a decrease in the total number of lymphocytes: from 1.0 to 1.5? 10 9 /l. With lymphadenopathy and asymptomatic infection, lymphopenia is observed in 40% of cases. In immunological research, great importance is attached to changing the normal ratio of helpers to suppressors. In healthy people, helpers account for 60% of T-lymphocytes. With manifest (manifest) AIDS, the ratio of helpers to suppressors is always below 1. With lymphadenopathy, a ratio of less than 1 is observed in 55%. The degree of immunodeficiency is judged by the ratio of helpers and suppressors.

To determine cellular immunity, an intradermal test is used. This is a multitest using 7 antigens and a control. In healthy people, there are at least two positive skin reactions (with a diameter of more than 10 mm in men, more than 5 mm in women). In patients with manifest forms of AIDS and in patients with lymphadenopathy, in almost all cases there is hyperergy or anergy. In asymptomatic carriers, hyperergy occurs in 20–40%. The change in humoral immunity is that in 50–60% of patients with manifest AIDS and in 30–40% of patients with lymphadenopathy, the content of JgA and JgJ is increased. In AIDS, the humoral response is qualitatively insufficient: B-lymphocytes react incompletely to microbial antigens, that is, they do not produce enough antibodies. This circumstance complicates the serological diagnosis of opportunistic infections. Additional tests include an increase in acute phase proteins, an increase in the level of low molecular weight protein in the serum? 2-microglobulin. The results of immunological tests should be assessed taking into account the characteristics of reactions in individual age groups. For example, in children, changes in the ratio of T-helper and T-suppressor cells for the diagnosis of AIDS are less important than in adults. This is due to the fact that deviations from the norm in children are less pronounced. In children, AIDS can be differentiated from congenital immunodeficiencies by polyclonal hypergammaglobulinemia. In general, immunological tests are considered as one of the important components complex diagnostics AIDS. Isolation of the causative agent of AIDS (human immunodeficiency virus - HIV) made it possible to specifically diagnose the disease. Specific laboratory diagnostics follow the following line:

1) virus detection;

2) detection of virus components (antigens, nucleic acid, reverse transcriptase);

3) detection of antibodies.

It should be noted that, although rare (0.2% of cases), false positive reactions are also possible. Therefore, serological tests, like other diagnostic methods, should be evaluated only in conjunction with other data. According to scientists, the most important test for serological diagnosis of AIDS is the enzyme-labeled antibody test (EMA). All positive and questionable sera must be tested by other complex tests that are based on different principles. Improving the reactions of enzyme-labeled antibodies makes it possible to avoid false-positive reactions, therefore, preventing errors when making a final diagnosis of AIDS.

Having examined a wide group of immunodeficiency conditions, we should conclude that, despite the universality of the body’s defense system that nature has created, it is not absolute, but only adapted to a certain set of natural conditions, the level and lifestyle of a particular person, which correspond to the individual norm of adaptation. Since human living conditions change, new environmental factors appear, the body is forced to adapt. Adaptation occurs even when changes in conditions correspond to the limits of adaptation inherent in the genotype. And such an adaptation necessarily includes mechanisms of adaptation and compensation, i.e., in other words, it can cause a pathological reaction of the body.

Immune status (IS) is a set of quantitative and functional indicators that reflect the state of the human immune system at a given time. This concept was introduced to objectively assess the state of the human immune system. The study of immunity parameters in immune disorders should include studies of the quantity and functional activity of the main components of the immune system. All dysfunctions of the immune system are classified based on the manifestations of various diseases. There are primary and secondary immunodeficiencies, autoimmune, allergic and lymphoproliferative diseases.

To assess the functioning of all components of the immune system, the T and B immune systems, phagocytic and complement systems, quantitative and functional methods must be included. To assess the humoral component of immunity, the following studies are performed: determination of the production of immunoglobulins of different classes in blood serum; determination of the relative and absolute content of B-lymphocytes and their subpopulations, complement components and circulating immune complexes, functional tests (blast transformation reaction with mitogens), determination of specific antibodies, skin tests.

To assess the T-cell link, studies are carried out to determine the relative and absolute number of T-lymphocytes and their subpopulations (T-helpers, CTLs), natural killer cells, their activation markers, functional tests (blast transformation reaction with mitogens), and determination of cytokine production.

The state of the phagocytic system is assessed using many tests: the adhesive ability of neutrophils to adhere to nylon fibers; migration, chemotaxis in the reaction of inhibition of neutrophil migration; metabolic activity and formation of reactive oxygen species for the reduction of nitroblue tetrazolium; phagocytic activity of neutrophils in tests of spontaneous and stimulated by microbial polysaccharides phagocytosis; immunophenotyping of neutrophils.

Previously, these methods were divided into Level 1 and Level 2 tests. Level 1 tests are indicative and are aimed at identifying gross defects in the immune system. Level 2 tests are aimed at identifying a specific “breakdown” in the immune system.

Level 1 tests

• determination of the relative and absolute number of leukocytes, neutrophils, monocytes, lymphocytes and platelets in peripheral blood;
• determination of functional activity of neutrophils (NST test);
• immunophenotyping tests to determine the relative and absolute number of T- and B-lymphocytes, natural killer cells;
• determination of the concentration of immunoglobulins of the main classes (IgA, IgM, IgG, IgE);
• determination of hemolytic activity of complement.

Using a minimal set of tests, you can diagnose primary immunodeficiencies: chronic granulomatous disease, X-linked agamma globulinemia, hyper-IgM syndrome, selective IgA deficiency, Wiskott-Aldrich syndrome, severe combined immunodeficiency.

Level 2 tests

• immunophenotyping tests to determine the relative and absolute number of populations and subpopulations of T-, B-, NK-lymphocytes;
• lymphocyte activation markers;
• assessment of the various stages of phagocytosis and the receptor apparatus of phagocytic cells;
• determination of the main classes and subclasses of immunoglobulins;
• circulating immune complexes;
• determination of the concentration of complement components in blood serum (C3, C4, C5, C1 inhibitor);
• functional activity of various subpopulations of lymphocytes;
• assessment of the proliferative activity of T- and B-lymphocytes;
• study of interferon status;
• skin tests, etc.

The set of indicators obtained during an immunological examination is called immunogram.

It should be especially emphasized that a full analysis of the immunogram is possible only in combination with the clinical condition and medical history of the patient. The absence of characteristic changes in the immunogram with pronounced clinical symptoms should be considered an atypical reaction of the immune system, which is an aggravating sign of the disease. The patient data obtained is compared with the average values ​​for that analyte obtained in the patient's region of residence. Average statistical indicators vary depending on the region and are subject to climatic and geographical conditions, environmental conditions, and living conditions. The patient's age and circadian rhythms must also be taken into account.

The study of IS indicators is of great importance for diagnosis and differential diagnosis, especially in primary immunodeficiencies and lymphoproliferative diseases, for assessing the severity, activity, duration and prognosis of various diseases, and assessing the effectiveness of treatment.

The immune system helps our body resist negative influences, serious diseases, and block different processes associated with the development of tumors. When it malfunctions, there can be serious health problems, and to identify and eliminate “weak spots” of the immune system, there is a special blood test that allows one to study the immune status and shows the patient’s body’s ability to resist various infections.

When an immunogram is done in the laboratory, several tests of the main indicators are carried out at once, clarifying the status of individual parameters.

• Determination of antibodies of various classes demonstrates the presence of infections in the body and the level of their development. By looking at the status of different groups, you can determine the duration of infection and draw a conclusion about the course of the disease.
• Determination of lymphocyte subpopulations makes it possible to determine the composition of each of the two existing groups lymphocytes, and notice their possible shortage.
• An analysis of the phagocytic activity of leukocytes shows the activity of phagocytosis - the process of absorption of bacteria and harmful viruses to prevent their influence on the body.
• C3 and C4 complement components are proteins from the complement system that play an important role in the process of inflammation, facilitating phagocytosis.
• Analysis for CIC (circulating immune complexes) examines the antigen-antibody chain, which is formed as an immune response to the ingress of foreign microorganisms.

Blood analysis

When an immunogram is done, they mainly use blood taken from a finger or from a vein. The collected amount of blood is distributed into two test tubes, in one of which the blood immediately coagulates and contains the molecules needed for analysis and a clot containing formed cells; another tube contains a substance that prevents blood from clotting, so that the necessary cells are preserved in the form of a suspension.

If the doctor is interested in the immune status of the mucous membranes, saliva, mucus or tear fluid is taken for analysis. If you need to find out the immune status of the nervous system, they take cerebrospinal fluid (CSF), but this happens in rare cases.

Indications for a blood test for immunity

If there is a disease viral origin, allergic reactions, frequent pneumonia, long-term fungal infections, inflammatory chronic pathologies (bronchitis, sinusitis), autoimmune diseases (diabetes mellitus, etc.), oncology, pustular skin pathologies, secondary and primary immunodeficiencies, diseases of the gastrointestinal tract of infectious origin, in which weight loss occurs, if you are after chemotherapy or after an organ transplant, you need to check your immune status.

There are also separate indications for a blood test for pregnant women if they have HIV, frequent relapses of herpes simplex, autoimmune pathologies, pregnancy with Rhesus conflict, continuous relapses cytomegalovirus infection, pathologies of tissue interaction during pregnancy.

Immune status – normal/not normal

The blood test is deciphered only by an immunologist, and not just by a layman or a familiar nurse, since it looks like a long list of abbreviations with corresponding numbers that are understandable only to a specialist.

If the immune status, visible from a blood test, shows that most of the indicators are normal, with the exception of a few things, you will be asked to donate another portion of blood in 1.5-3 weeks to compare the results and obtain a more accurate diagnosis. If a blood test demonstrates a decrease in the level of phagocytes and their functional activity, a suppurative process may be present. When a T-lymphocyte defect can be observed, AIDS is most likely diagnosed. If the norm of IgE immunoglobulins is exceeded, one can judge helminthic infestations or allergies, and if

1. The concept of immune status

2.

3.

4. Methods for assessing immune status

1. State of functional activity of the human immune system as a whole has vital importance for the body and is designated by the concept "immune status".

Immune status - This quantitative and qualitative characteristics of the state of functional activity of the immune system organs and some nonspecific mechanisms of antimicrobial defense.

Disorders of the immune status and the ability to produce a normal immune response to various antigens are called immunodeficiency states (immunodeficiencies), who share.

For primary (congenital, hereditary);

Secondary (acquired).

2. Primary human immunodeficiency- genetically determined inability of the body to implement one or another link of immunity. They appear soon after birth and are inherited, as a rule, in a recessive manner.

Primary immunodeficiency conditions can be expressed in damage to the B- and T-system of immunity and auxiliary cells (antibody formation and cellular forms) of the immune response, and may be combined, but they are all called specific, in contrast to hereditarily determined defects in nonspecific protective factors - phagocytosis, complement system, etc.

The most characteristic clinical manifestation of primary immunodeficiency states is recurrent infections upper respiratory tract and digestive tract, pyoderma, arthritis, osteomyelitis.

In case of insufficiency humoral immunity prevail bacterial infections; in case of insufficiency cellular - viral and fungal.

3. Secondary immunodeficiency conditions arise as a consequence of immunoregulation disorders and other pathological processes, accompanied lymphopenia And hypogammaglobulinemia.

Secondary immunodeficiencies are associated with the following circumstances:

Previous infectious diseases (measles, influenza, leprosy, candidiasis);

Somatic (with nephrotic syndrome);

Oncological (tumors of lymphoreticular nature) diseases;

Burns;

Severe injuries;

Extensive surgical interventions;

Some therapeutic effects (X-ray irradiation, radiation therapy tumors, therapy with corticosteroids, cytostatics and immunosuppressants during tissue and organ transplantation, thymectomy, splenectomy, etc.).

For chronic lymphocytic leukemia, myeloma, macroglobulin-mia and diseases accompanied loss of protein mostly suffers B-immune system.

For lymphogranulomatosis, Hodgkin's disease, leprosy, viral infections - T-system.

Old age is a pronounced T-immunodeficiency.

4. To identify immunodeficiency states, there is a need to assess indicators of the functional activity of the immune system, i.e. immune status. Assessment of immune status consists of several stages:

clinical and laboratory, which includes:

Collection and assessment of immunological history (frequency of infectious diseases, nature of their course, severity of temperature reaction, presence of foci of chronic infection, reactions to vaccinations or administration of medications);

Evaluation of the results of a general clinical blood test (content of granulocytes, monocytes, lymphocytes);

Detection of bacterial and viral carriage using bacteriological, virological and/or serological studies;

laboratory-immunological. At this stage, studies are carried out in the immunological laboratory, the purpose of which, in fact, is the qualitative and quantitative assessment of the functional activity of the immune system (immune competent cells). For this purpose, a series (set) of tests has been developed, which are divided into tests of the 1st (indicative) and 2nd (analytical) levels.

Level 1 tests are indicative and allow you to identify gross violations of the immune system.

They include definition:

Total and relative number of lymphocytes;

Main subpopulations (T and B cells);

Phagocytic activity of leukocytes;

Concentrations of immunoglobulins of different classes in blood serum.

The total (absolute) and relative number of lymphocytes is determined according to the data clinical blood test. The content of T- and B-lymphocytes is calculated in immunofluorescence reactions, using labeled monoclonal fluorescent sera to specific surface antigen markers, denoted by CD symbols (cluster differentiation). Several dozen such antigenic markers are known, but some of them are characteristic of one or another type of cell:

CD3 receptor - all T lymphocytes;

Receptors CD19, 20, 21, 72 - B lymphocytes;

CD4 receptors - T helper cells;

CD8 receptors - T-suppressors;

CD16 receptors are NK cells (natural killer cells).

More accessible and simpler, but less accurate and outdated is rosette formation method. It is based on the fact that B lymphocytes can adsorb mouse erythrocytes on their surface, and T lymphocytes can adsorb sheep erythrocytes (they can also be formed by NK cells). A lymphocyte with red blood cells stuck to it - this is socket, they are counted in colored according to Romanovsky-Giemsa smears from a mixture of lymphocytes and corresponding red blood cells.

To assess the phagocytic activity of blood neutrophils, determine percentage of phagocytic cells And phagocytic indicator(average number of microbial cells absorbed by one leukocyte).

The concentration (level) of immunoglobulins of different classes G, M, A and E in blood serum is determined in gel precipitation reactions (radial immunodiffusion according to Mancini) with anti-globulin sera to IgG, IgM, IgA, IgE, but this method gives a fairly large error in determination: ± 15%.

Level 2 tests allow for a more in-depth analysis of the state of the immune system and clarify the nature of the defects identified using level 1 tests. These include, for example, the determination of individual subclasses of immunoglobulins (especially IgG, secretory IgA) and B lymphocytes, regulatory and effector cells.

In addition, using immunoenzyme and radioimmune methods can determine the concentrations of individual cytokines - the main regulatory molecules that determine the type of immune response.

For example, interleukin-2 is an essential component of the immune system I a strong response to any antigens, including microbial ones, as it ensures the proliferation and differentiation of T-lymphocytes.