When immunodeficiency occurs. Primary immunodeficiencies

Dangerous disease, difficult to treat – secondary immunodeficiency. It is not a consequence of genetic predisposition and is characterized by a general weakening of the body and immune system. Secondary immunodeficiencies are defined by immunology as acquired pathological disorder at work protective forces our body.

What does secondary immunodeficiency mean?

If we consider secondary immunodeficiency in more detail, what it is in adults, we can give a definition formulated in the section general medicine, which studies the protective properties of the body and its resistance to external factors - immunology. So, secondary (acquired) immunodeficiency is a malfunction of the immune system that is in no way related to genetics. Such conditions are accompanied by various inflammatory and infectious diseases that are very difficult to treat.

Secondary immunodeficiencies - classification

There are several types of classification of such conditions:

by speed of development;
by prevalence;
by level of breakdown;
according to the severity of the condition.

Classification of secondary IDS according to the rate of progression:

acute (caused by acute infectious diseases, various toxicities, injuries);
chronic (appears against the background of autoimmune disorders, viral infection, tumors, etc.).

By level of breakdown:

secondary immunodeficiency at the phagocytic level;
complement system defect;
secondary T-cell immunodeficiency;
violation of humoral immunity;
combined.

They also highlight:

spontaneous IDS – similar to primary immunodeficiency because there is no obvious reason emergence;
secondary induced immunodeficiency syndrome - the cause of which is clear.

Forms of secondary immunodeficiencies

In addition to the classifications considered, secondary acquired immunodeficiencies are also distinguished, spontaneous and induced. You can often find AIDS as one of the forms of this condition, but modern immunology more often mentions this syndrome as a consequence of acquired IDS, the causative agent of which is HIV (human immunodeficiency virus). AIDS, along with the spontaneous and induced forms, are combined into a single concept - secondary acquired immunodeficiency.

Spontaneous form of secondary immunodeficiency

The absence of a specific, obvious etiology characterizes spontaneous immunodeficiency. This makes it similar to primary species, and more often it is caused by exposure to opportunistic microbiota. In adults, chronic inflammation that is difficult to treat is defined as the clinical manifestations of secondary IDS. The most common infections are observed in the following organs and systems:

eyes;
skin;
organs of the respiratory system:
gastrointestinal organs;
genitourinary system.

Induced secondary immunodeficiency

Induced immunodeficiency is treatable and often with complex therapy it is possible to completely restore the functioning of the body's defenses. The most common reasons why secondary induced immunodeficiency occurs are:

surgical interventions;
serious injuries;
pathologies due to diabetes, liver and kidney diseases;
frequent x-rays.

Causes of secondary immunodeficiencies

There are many reasons that cause secondary immunodeficiency syndrome and the average reader does not even know about many of them, because most people associate the concept of IDS with something global and irreversible, but in fact such conditions are reversible, if we are not talking about the immunodeficiency virus person. But even if we talk about HIV, many live to a ripe old age with this virus.

So, the reasons for the appearance of such conditions may be:

bacterial infection (tuberculosis, pneumococci, staphylococci, meningococci, and so on);
helminths and protozoal infestations (roundworms, toxoplasmosis, trichinosis, malaria);
oncological education.
autoimmune problems.
viral infections (smallpox, hepatitis, measles, rubella, herpes, cytomegaly, and so on);
intoxication (thyrotoxicosis, poisoning);
severe psychological and physical injuries, increased physical activity;
bleeding, nephritis, burns;
chemical influences (drugs, steroids, chemotherapy);
natural factors (old age or childhood, period of pregnancy);
lack of important micro and macroelements and vitamins due to poor nutrition.

Secondary immunodeficiency - symptoms

Symptoms, which often indicate the presence of problems, can be a signal for an immediate examination of the immune system. Signs of secondary immunodeficiency:

The question of how to treat secondary immunodeficiency requires detailed consideration, because not only immunity, but also, often, life depends on therapy. At frequent illnesses against the background of low immunity, you should urgently consult a specialist and undergo an examination. If secondary immunodeficiency has been diagnosed, then you should not delay the start of treatment.

Treatment of secondary ISD is prescribed depending on the link in which the breakdown is detected. During therapy, measures are taken first to eliminate the causes of the disease. As a rule, these are the correct recreational measures after operations, injuries, burns, etc. If the body becomes infected, the presence of bacteria, viruses, and fungi will be eliminated with the help of medications.

For infections caused by pathogenic bacteria, it is prescribed antibiotic drugs(Abactal, Amoxiclav, Vancomycin, Gentamicin, Oxacillin).
If pathogenic fungi have been detected, then prescribe antifungal agents(Ecodax, Candide, Diflucan, Fungoterbin).
Anthelmintic drugs are prescribed in the presence of worms (Helmintox, Zentel, Nemozol, Pirantel).
Antiviral and antiretroviral drugs are prescribed for human immunodeficiency virus (Amiksin, Arbidol, Abacavir, Phosphazide).
Immunoglobulin injections intravenously are used when the body’s production of its own immunoglobulins is reduced (Normal human immunoglobulin, Hyperimmunoglobulin).
Immunocorrectors are prescribed for various acute and chronic infections (Corditsex, Roncoleukin, Yuvet, etc.).

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Secondary immunodeficiency is a condition characterized by reduced functionality of the immune system. When this disease appears, a person’s resistance to various types of infections decreases. Secondary immunodeficiency is a common phenomenon; it occurs much more often than primary immunodeficiency. Secondary, as a rule, can be corrected, but only if the person does not have HIV infection.

This pathological condition does not occur on its own; bacterial and viral infections play a role in its formation.

Weakened immunity in a child

Primary immunodeficiency in children can appear immediately after birth; secondary immunodeficiency develops after one or another part of the immune system has been damaged. The disease manifests itself in the form of recurrent infections, along with which tumors often form in the child. The manifestation of immunodeficiency can be of an allergic nature. Primary and secondary immunodeficiencies occur in children. Secondary is the result of exposure to some external stimulus; As for the primary one, it is rare in children (mostly inherited). With primary immunodeficiency, the child is born unhealthy. The cause of secondary immunodeficiency may be prematurity, Down syndrome, HIV infection, hematological diseases, injuries, serious surgical interventions.

Provoking factors

The classification of secondary human immunodeficiencies has its own characteristics. It is worth knowing that the immune system is complex structure, it can easily fail, as a result of which its mechanism will be disrupted. The causes of secondary immunodeficiency are conventionally divided into two groups: external and internal. As for external ones, these should include such as frequent overwork, stress, being in the cold, lack of proper sanitary conditions, failure to comply with personal hygiene rules, poor nutrition. Excessive exposure to ultraviolet radiation can also cause weakened immunity.

Secondary immunodeficiency can occur in people (including children) who have been taking antibiotics, glucocorticoids and other drugs that can provoke the disease for a long time. Taking these drugs may have negative impact on the state of the body, along with which the immune system suffers. If influence is exerted external factors, the disease does not appear immediately, but gradually. It is worth remembering that all organs and systems are interconnected; if a person suffers from severe immunodeficiency, the functioning of all organs is significantly impaired.

As noted above, there are internal factors that contribute to the development of the disease, these include rubella, viruses, causing herpes, malaria, toxoplasmosis, leishmaniasis. If a person suffers from a chronic infectious disease, the reactivity of his immune system decreases and his susceptibility to pathogenic microbes increases. During the course of chronic infectious disease intoxication of the body occurs. TO internal factors Secondary immunodeficiency should include formations of a malignant type, in the case of which there is a disruption in the functioning of organs. If we talk about the most pronounced decrease in immunity, we should mention malignant diseases blood (leukemia). Against the background of leukemia, symptoms of immunodeficiency are always observed.

Vitamin deficiency and endocrine diseases

A decrease in immunity can be caused by the fact that a person has not eaten properly. It is worth knowing that the immune system clearly reacts to a lack of vitamins. If the body experiences a lack of vitamins, microelements and nutrients, his protective functions are weakened. IN frequent cases the patient experiences a lack of vitamins during a certain period of time, for example, during seasonal vitamin deficiency. The immune system is weakened if a person has lost a lot of blood and nutrients. It is worth remembering that any serious illness may cause weakening protective properties organism, and therefore the appearance of primary and secondary immunodeficiency cannot be ruled out. Secondary can be provoked by hormones called “Adrenal glands”; as a result of their influence, the protective properties of the immune system are suppressed.

If a person suffers endocrine diseases, this also leads to weakened immunity. A typical example is diabetes mellitus, in which there is a decrease in energy production in the tissues. If the patient suffers diabetes mellitus, the likelihood of other diseases increasing. The victim's blood contains large number glucose, this negatively affects the functioning of the immune system. Elderly people may have physiological immunodeficiency, which is due age-related changes in the body.

Pathology that is rare

Common variable immunodeficiency is a fairly rare type of disease that can occur in people of any age. An illness of this nature occurs in teenagers and young men under 20 years of age. Symptoms of variable immunodeficiency manifest themselves in different ways. A person may have bacterial infection skin, chronic infection, dysbacteriosis. With this disease, a person’s spleen enlarges, and symptoms may appear. malignant formations in the gastric zone. When a person is in this state, he experiences rapid development of autoimmune diseases. Variable immunodeficiency has a nature that is not fully understood, scientists suggest that important role Hereditary predisposition plays a role in the development of the disease.

To identify the disease, it is necessary to conduct a diagnosis. To confirm the diagnosis, all family members of the person suspected of the disease must be diagnosed. Variable immunodeficiency sometimes occurs in pet, in this case, you cannot do without the help of a veterinarian. As for the duration of the disease, it should be noted that it lasts a lifetime, during which time the patient needs treatment. A person constantly needs injections of immunoglobulins, which must be administered intravenously. If a bacterial infection manifests itself, antibiotics will be needed to overcome it. A person needs urgent medical assistance if he has constant recurrent infections that have bacterial nature. Seeing a doctor is necessary for diarrhea, the causes of which cannot be explained.

How is the disease diagnosed?

Secondary immunodeficiency is a disease that can occur in people of any age. If a person treats an illness, it subsides, and then worsens again, you should seriously think about it and seek help again. A problem can be suspected if medications against a disease are ineffective. Another sign of the disease is an increase in body temperature. A person may have increased fatigue, frequent occurrence of infectious diseases, fatigue of a chronic nature. To determine congenital immunodeficiency, it is necessary to conduct several tests, during which abnormalities will be visible. The diagnosis of the disease can only be made after the patient will pass comprehensive examination and will receive advice from a doctor (the diagnosis is made after an assessment based on immune status).

The immune status is determined only by a doctor; in the process, a detailed study of the activity of the components of the immune system takes place; they play a key role in the body’s resistance to infections. Patients who have been diagnosed with the disease can be divided into three groups. The first contains patients who have been diagnosed various signs immune deficiency, the immune status parameter is changed. In the second group are people who have signs of immune deficiency, but their immune status is normal. The third group includes patients who have changes in immune status, but there are no signs of immune deficiency.

For the first two groups, it is necessary to prescribe immunotropic treatment, which will help identify and correct disorders. Patients of the third group need a thorough examination, as a result of which the causes of the disease will be clarified.

If a person, including a child, experiences secondary immunodeficiency, the disorders are less pronounced than in the case of primary immunodeficiency. The disease can be cured with the restoration of all necessary protective properties. Treatment should begin after the causes of the disease are identified.

Drugs to treat illness

Used to treat immunodeficiency various means. If the disease occurs against the background of infections that have chronic nature, it is necessary to begin therapy with sanitation of lesions chronic inflammation. If the disease occurs as a result of vitamin deficiency, it must be treated with another method. As a rule, the doctor prescribes medications containing vitamins, minerals, and various beneficial substances; in addition, medications that are added to food may be prescribed. To eliminate secondary immunodeficiency, immunotropic treatment is often used. In order for the patient to recover as soon as possible, the doctor prescribes a course of treatment with drugs that stimulate the properties of the immune system (each of them has its own mechanism of action). For any manifestations of the disease, self-medication is prohibited.

During the treatment process, it is important to take into account the degree of the disease and all its features. Vaccination is used only during remissions of various infectious and somatic diseases. It is worth remembering that the drugs used have their own characteristics and indications for use. In some cases it is revealed individual intolerance one medicine or another. Interferons and intravenous immunoglobulins often used to treat patients with secondary immunodeficiency. The prescription of a particular drug has a number of features. Immunomodulators are prescribed during the stages of remission of the infectious process. The prescription of an immunomodulator directly depends on the severity of the disease and the cause of its occurrence. Immunomodulators are prescribed for manifestations of immunodeficiency. Doses, regimens and duration of therapy must comply with the instructions for the drug; correction of drug use regimens should be carried out by an immunologist.

The success of treatment of primary immunodeficiency, as well as secondary, depends on the experience and professionalism of the immunologist. His tasks include drawing up a treatment regimen that will help overcome the disease.

IDS (immunodeficiency states) are pathologies, the course of which is characterized by a decrease in activity or a complete inability of the body to perform functions immune defense.

Classification of immunodeficiency states

Based on their origin, all immunodeficiency states are divided into three main groups:

1. Physiological.

2. Primary immunodeficiency states. May be congenital or hereditary. Primary immunodeficiency conditions result from genetic defect, under the influence of which the processes of functioning of the cells of the immune system of the human body are disrupted.

3. Secondary immunodeficiency states (acquired after birth, during life). They develop under the influence of various biological and physical factors.

Based on the primary damage to the cells of the body’s immune system, immunodeficiency states are divided into 4 groups:

with damage to humoral immunity (humoral, B-dependent);
with damage cellular immunity(cellular, T-dependent);
with damage to phagocytosis (A-dependent);
combined (when both humoral and cellular immunity are affected).

Causes of immunodeficiency conditions

Since there are quite a lot of reasons for the violation of the body’s immune defense, they are conditionally divided into several groups.

The first group includes congenital immunodeficiency conditions, when the disease manifests itself immediately after birth or in early childhood.

The second group includes secondary immunodeficiency conditions, the cause of which may be:

Both acquired and congenital immunodeficiency conditions have similar clinical signs:

Increased susceptibility to any infections;

Aches and pain in muscles, bones, joints;

Frequent exacerbations of chronic diseases (arthrosis, arthritis, tonsillitis, respiratory diseases, gastrointestinal tract, and so on);

Painful and enlarged lymph nodes;

The combination of several pathologies in one disease of various etiologies(fungal, bacterial, viral);

Constantly elevated temperature body (up to 37.7 degrees);

Low effectiveness of taking antibiotics;

General weakness, causeless fatigue, lethargy, depressed mood;

Increased sweating.

In addition to the clinical picture, when making a diagnosis, it is necessary to identify the cause of immunodeficiency. This is necessary in order to develop the correct treatment regimen and not cause even more harm to the body.

Immunodeficiency conditions in children

IDS in children develop as a result of damage to one or several parts of the body’s immune defense.

Immunodeficiency states manifest themselves in children in the form of severe and often recurrent infections. It is also possible to develop tumors and autoimmune manifestations against the background of immunodeficiency.

Some types of IDS in children manifest themselves, including in the form of allergies.

There are two types of immunodeficiency in children: primary and secondary. Primary immunodeficiencies are caused by genetic changes and are less common than secondary ones, which are caused by external influence or any disease.

Diagnosis of immunodeficiency conditions

When making a diagnosis, the doctor first of all pays attention to the family history. He finds out if there have been cases in the family autoimmune diseases, early death, cancer diseases in relatively at a young age and so on. Another sign of IDS may be adverse reaction for vaccination.

After the interview, the doctor begins the examination. It is necessary to pay attention to appearance patient. Typically, a person suffering from immunodeficiency has sickly look. He has pale skin, which often has various types of rashes. Man complains about constant weakness and overwork.

In addition, his eyes may be inflamed and chronic diseases ENT organs, persistent cough, swelling of the nostrils.

To clarify the diagnosis, carry out additional examination which may include:

blood test (general, biochemical);
urine test;
screening tests;
determining the level of immunoglobulins in the blood and so on.

If it turns out that the patient has a recurrent infection, then measures are taken to eliminate it. If necessary, smears can be taken and then examined under a microscope to identify the causative agent of infection.

Treatment of immunodeficiency conditions

Immunodeficiency conditions are treated with antibacterial, antiviral, antifungal drugs, as well as immunomodulators.

Taking into account which part of the immune defense has been compromised, drugs such as interferon, echinacea herb, Taktivin, and so on can be prescribed.

Treatment for bacterial infection immunodeficiency states includes admission antibacterial drugs wide range actions in combination with immunoglobulins.

At viral diseases purpose shown antiviral agents(Valtrex, Acyclovir and a number of others).

A diet must be prescribed (with an emphasis on protein food), oxygen baths, vitamin therapy. Physical activity is indicated. In some cases, a transplant is performed bone marrow.

There are medications that must be used if the immune system is weak. They are called immunomodulators. Perhaps the most famous and effective drug from this group is Transfer Factor.

This most powerful immunomodulator a new generation, which, when entering the patient’s body, has the following effect:

quickly restores the functioning of the immune system, normalizes metabolic processes;
has a potentiating effect, enhancing therapeutic effect from concomitantly taken medications;
blocks possible side effects from other drugs;
"remembers" pathogenic microorganisms and when they subsequently enter the body, it gives a signal for their immediate destruction.

Transfer Factor is a 100% natural composition, so it does not give side effects and has virtually no contraindications.

Prevention of immunodeficiency conditions

In order to reduce the likelihood of developing IDS, the following recommendations must be followed:

1. Eat right. This is one of the main rules for strengthening the body's immune defense. Enthusiasm junk food leads to violation metabolic processes, which, in turn, triggers a cascade of reactions that weaken the immune system.

Here are some principles of rational nutrition:

food should be multi-component and varied;
you need to eat fractionally (5-6 times a day, in small portions);
you need to combine less fats and proteins, different types proteins, proteins and carbohydrates, acidic foods with proteins and carbohydrates, bread and pasta with vegetables or fats;
maintain the following ratio: fats - 20%, proteins - 15%, carbohydrates - 65%;
it is necessary to limit the consumption of sweets, flour, canned food, sausages, smoked meats, store-bought juices (they contain a lot of sugar and little useful substances), soda, salt, chocolate, coffee.

2. To maintain beneficial microflora Gastrointestinal tract must be consumed special means, prebiotics and probiotics. They contain about 80% of all immune cells body. Weak immunity often occurs precisely because of a violation of the amount of beneficial microflora in the intestines. The most powerful and effective probiotics are:

Santa Rus';
Vetom;
Kutushov's symbionts;
Unibacter;
Acidophilus and a number of others.

3. News healthy image life. It is known that weak immunity in our time, it is often a consequence of physical inactivity. Modern man spends a lot of time in the office or at the computer, so he moves around and is fresh air much less frequently than his ancestors. Therefore, it is vital to compensate for the lack motor activity playing sports and walking more often.

4. Temper the body. By the best means for this purpose are contrast shower and a bathhouse.

5. Avoid nervous overstrain and stress.

I wish you health and strong immunity!

Immunodeficiency conditions

Numerous immunodeficiency states have been found in humans. Their classification is given in the table. People suffering from immunodeficiency have a high incidence of malignant tumors and autoantibodies (the latter may be accompanied by autoimmune diseases). This is due to dysregulation of T-cell activity or the body's inability to cope with underlying viral diseases.

Table. Classification of immunodeficiency states

Type of immunodeficiency		Infectious agent
Complement system deficiency	Complement component C3 deficiency	Pyogenic bacteria	Antibiotics
Myeloid cell deficiency	Chronic granulomatosis	Bacteria containing catalase	Antibiotics
B cell deficiency	Infantile sex-linked agammaglobulinemia (Bruton's disease)	Pyogenic bacteria Pneumocystis carinii	Gamma globulin
T cell deficiency	Thymic hypoplasia	Candida, viruses	Thymus transplantation
Stem cell deficiency	Severe combined deficiency (Swiss type agammaglobulinemia)	All above	Bone marrow transplantation

Primary immunodeficiency states occur in humans (albeit quite rarely) as a result of disruption of almost any stage of differentiation of cells of the immune system. Deficiencies of the complement system, phagocytes, or B cells lead to bacterial infections that the body normally deals with through opsonization and phagocytosis. T-cell deficiency causes increased sensitivity of the body to viruses and fungi, the destruction of which is based on cellular immune reactions.

Secondary immunodeficiency states can also develop as a consequence of malnutrition, lymphoproliferative diseases, viral infections, X-ray irradiation and exposure to cytotoxic drugs. medicines. The most dangerous disease at present is acquired immunodeficiency syndrome (AIDS). This disease is caused by a retrovirus (human immunodeficiency virus, HIV), which selectively attacks T-helper cells necessary for the functioning of the cellular immune system. A person with AIDS becomes defenseless against infections caused by opportunistic microorganisms (in particular, Pneumocystis carinii and cytomegalovirus), which are fatal. If virus-neutralizing antibodies are found in the blood of AIDS patients, they are in low titers.

Immunodeficiencies (IDS) are disorders of immunological reactivity caused by the loss of one or more components of the immune apparatus or nonspecific factors closely interacting with it.

Primary immunodeficiencies are congenital (genetic or embryopathies) defects of the immune system. Depending on the level of violations and localization of the defect, they are:

humoral or antibody - with predominant damage to the B-lymphocyte system)

X-linked agammaglobulinemia (Bruton's disease)

Hyper-IgM syndrome

X-linked

autosomal recessive

deletion of immunoglobulin heavy chain genes

k-chain deficiency

selective deficiency of IgG subclasses with or without IgA deficiency

antibody deficiency with normal immunoglobulin levels

common variable immune deficiency

IgA deficiency

cellular

DiGeorge syndrome

primary CD4 cell deficiency

CD7 T cell deficiency

IL-2 deficiency

multiple cytokine deficiency

signal transmission defect

combined:

Wiskott-Aldrich syndrome

ataxia-telangiectasia (Louis-Bar syndrome)

severe combined immune deficiency

X-linked to the floor

autosomal recessive

adenosine deaminase deficiency

purine nucleoside phosphorylase deficiency

deficiency of MHC class II molecules (bald lymphocyte syndrome)

reticular dysgenesis

CD3γ or CD3ε deficiency

CD8 lymphocyte deficiency

complement deficiency

phagocytosis defects

hereditary neutropenia

infantile lethal agranulocytosis (Kostman's disease)

cyclic neutropenia

familial benign neutropenia

defects in phagocytic function

chronic granulomatous disease

X-linked

autosomal recessive

type I lymphocyte adhesion deficiency

leukocyte adhesion deficiency type 2

Neutrophil glucose-6-dehydrogenase deficiency

myeloperoxidase deficiency

deficiency of secondary granules

Shwachman syndrome

Clinical picture of IDS

The clinic has a number of common features:

1. Recurrent and chronic infections of the upper respiratory tract, paranasal sinuses, skin, mucous membranes, gastrointestinal tract, often caused by opportunistic bacteria, protozoa, fungi, tending to generalize, septicemia and torpid to conventional therapy.

2. Hematological deficits: leukocytopenia, thrombocytopenia, anemia (hemolytic and megaloblastic).

3. Autoimmune disorders: SLE-like syndrome, arthritis, scleroderma, chronic active hepatitis, thyroiditis.

4. IDS is often combined with type 1 allergic reactions in the form of eczema, angioedema, allergic reactions for the administration of medications, immunoglobulin, blood.

5. Tumors and lymphoproliferative diseases with IDS are 1000 times more common than without IDS.

6. Patients with IDS often experience digestive disorders, diarrhea and malabsorption syndrome.

7. Patients with IDS have unusual reactions to vaccination, and the use of live vaccines in them is dangerous for the development of sepsis.

8. Primary IDS are often combined with developmental defects, primarily with hypoplasia of the cellular elements of cartilage and hair. Cardiovascular defects have been described mainly in DiGeorge syndrome.

Treatment of primary IDS

Etiotropic therapy consists of correcting a genetic defect using genetic engineering methods. But this approach is experimental. The main efforts with the established primary IDS are aimed at:

infection prevention

replacement correction of a defective part of the immune system in the form of bone marrow transplantation, immunoglobulin replacement, neutrophil transfusion.

enzyme replacement therapy

cytokine therapy

vitamin therapy

treatment of concomitant infections

Secondary immunodeficiencies

Factors that can cause secondary immunodeficiency are very diverse. Secondary immunodeficiency can be caused by both factors external environment, and internal factors of the body. In general, all unfavorable environmental factors that can disrupt the body's metabolism can cause the development of secondary immunodeficiency. The most common environmental factors that cause immunodeficiency include pollution environment, ionizing and microwave radiation, poisoning, long-term use of certain medications, chronic stress and fatigue. A common feature of the factors described above is a complex negative impact on all body systems, including the immune system. In addition, factors such as ionizing radiation have a selective inhibitory effect on the immune system associated with inhibition of the hematopoietic system. People living or working in a polluted environment are more likely to suffer from various infectious diseases and more often suffer from cancer. It is obvious that such an increase in morbidity in this category of people is associated with a decrease in the activity of the immune system.

Secondary immunodeficiencies are a common complication of many diseases and conditions. The main causes of secondary IDS:

Malnutrition and general exhaustion of the body also lead to decreased immunity. Against the background of general exhaustion of the body, the work of all internal organs is disrupted. The immune system is especially sensitive to deficiencies of vitamins, minerals and nutrients, since the implementation of immune defense is an energy-intensive process. Often a decrease in immunity is observed during seasonal vitamin deficiency (winter-spring)

helminthiasis

loss of immune defense factors is observed during heavy losses blood, burns or kidney disease (proteinuria, chronic renal failure). Common feature These pathologies are a significant loss of blood plasma or proteins dissolved in it, some of which are immunoglobulins and other components of the immune system (complement system proteins, C-reactive protein). During bleeding, not only plasma is lost, but also blood cells, therefore, against the background of severe bleeding, the decrease in immunity has a combined nature (cellular-humoral)

diarrhea syndrome

stress syndrome

Severe injuries and operations also occur with a decrease in the function of the immune system. In general, any serious disease of the body leads to secondary immunodeficiency. This is partly due to metabolic disorders and intoxication of the body, and partly due to the fact that during injuries or operations large amounts of adrenal hormones are released, which inhibit the function of the immune system

endocrinopathies (DM, hypothyroidism, hyperthyroidism) lead to a decrease in immunity due to metabolic disorders of the body. The most pronounced decrease in the body's immune reactivity is observed in diabetes mellitus and hypothyroidism. With these diseases, energy production in tissues decreases, which leads to disruption of the processes of cell division and differentiation, including cells of the immune system. Against the background of diabetes mellitus, the incidence of various infectious diseases increases significantly. This is due not only to the suppression of the function of the immune system, but also to the fact that increased content glucose in the blood of diabetic patients stimulates the growth of bacteria

Taking various medications and drugs has a pronounced immunosuppressive effect. The decrease in immune defense is especially pronounced when taking cytostatics, glucocorticoid hormones, antimetabolites, and antibiotics

low birth weight

a decrease in immune defense in elderly people, pregnant women and children is associated with age-related and physiological characteristics of the body of these categories of people

malignant neoplasms - disrupt the activity of all body systems. The most pronounced decrease in immunity is observed in the case of malignant blood diseases (leukemia) and when red bone marrow is replaced by tumor metastases. Against the background of leukemia, the number of immune cells in the blood sometimes increases tens, hundreds and thousands of times, but these cells are non-functional and therefore cannot provide the body’s normal immune defense

Autoimmune diseases occur due to dysfunction of the immune system. Against the background of diseases of this type and during their treatment, the immune system does not work enough and, sometimes, incorrectly, which leads to damage to its own tissues and the inability to overcome the infection

Treatment of secondary IDS

The mechanisms of immune suppression in secondary IDS are different, and, as a rule, there is a combination of several mechanisms; disorders of the immune system are less pronounced than in primary ones. As a rule, secondary immunodeficiencies are transient. In this regard, the treatment of secondary immunodeficiencies is much simpler and more effective compared to the treatment of primary disorders of the immune system. Typically, treatment of secondary immunodeficiency begins with identifying and eliminating the cause of its occurrence. For example, treatment of immunodeficiency against the background of chronic infections begins with the sanitation of foci of chronic inflammation. Immunodeficiency against the background of vitamin and mineral deficiency begins to be treated with the help of complexes of vitamins and minerals and various food additives(dietary supplements) containing these elements. The restorative abilities of the immune system are great, so eliminating the cause of immunodeficiency usually leads to restoration of the immune system. To speed up recovery and specifically stimulate the immune system, a course of treatment with immunostimulating drugs is carried out. At the moment, a large number of different immunostimulating drugs are known, with various mechanisms actions.

Autoimmune diseases are a class of diseases, heterogeneous in clinical manifestations, that develop as a result of the pathological production of autoimmune antibodies or the proliferation of autoaggressive clones of killer cells against healthy, normal tissues of the body, leading to damage and destruction of normal tissues and the development of autoimmune inflammation.

Possible reasons

The production of pathological antibodies or pathological killer cells may be associated with infection of the body with such an infectious agent, the antigenic determinants (epitopes) of the most important proteins of which resemble the antigenic determinants of normal tissues of the host body. It is by this mechanism that autoimmune glomerulonephritis develops after a streptococcal infection, or autoimmune reactive arthritis after gonorrhea.

An autoimmune reaction can also be associated with tissue destruction or necrosis caused by an infectious agent, or a change in their antigenic structure so that the pathologically altered tissue becomes immunogenic for the host organism. It is by this mechanism that autoimmune chronic active hepatitis develops after hepatitis B.

The third possible cause of an autoimmune reaction is a violation of the integrity of tissue (histo-hematological) barriers that normally separate some organs and tissues from the blood and, accordingly, from the immune aggression of the host lymphocytes. Moreover, since normally the antigens of these tissues do not enter the blood at all, the thymus normally does not produce negative selection (destruction) of autoaggressive lymphocytes against these tissues. But this does not interfere with the normal functioning of the organ as long as the tissue barrier separating this body from blood. It is by this mechanism that chronic autoimmune prostatitis develops: normally the prostate is separated from the blood by the blood-prostatic barrier, prostate tissue antigens do not enter the blood, and the thymus does not destroy “anti-prostatic” lymphocytes. But with inflammation, injury or infection of the prostate, the integrity of the blood-prostatic barrier is disrupted and auto-aggression against prostate tissue can begin. Autoimmune thyroiditis develops by a similar mechanism, since normally the thyroid colloid also does not enter the bloodstream (blood-thyroid barrier), only thyroglobulin with associated T3 and T4 is released into the blood.

The fourth possible cause of the body’s autoimmune reaction is a hyperimmune state (pathologically enhanced immunity) or an immunological imbalance with a violation of the “selective” function of the thymus that suppresses autoimmunity or with a decrease in the activity of the T-suppressor subpopulation of cells and an increase in the activity of killer and helper subpopulations.

Development mechanism

Autoimmune diseases are caused by dysfunction of the immune system as a whole or its individual components. In particular, it has been proven that suppressor T lymphocytes are involved in the development of systemic lupus erythematosus, myasthenia gravis or diffuse toxic goiter. In these diseases, there is a decrease in the function of this group of lymphocytes, which normally inhibit the development of the immune response and prevent aggression of the body’s own tissues. With scleroderma, there is an increase in the function of helper T-lymphocytes (T-helpers), which in turn leads to the development of an excessive immune response to the body's own antigens. It is possible that both of these mechanisms, as well as other types of dysfunction of the immune system, are involved in the pathogenesis of some autoimmune diseases.

Evolution

Most autoimmune diseases are chronic. In their development there are periods of exacerbations and remissions. Typically, chronic autoimmune diseases lead to serious violations functions of internal organs and disability of the patient. Autoimmune reactions that accompany various diseases or medications, on the contrary, are short-lived and disappear along with the disease that causes their development.

Immunosuppressants

Azathioprine

Infliximab

Prednisolone

Timodepressin

Cyclophosphamide

Biologically active agents (Considered the most promising)

TNF-α blockers (Infliximab, Adalimumab, Etanercept)

CD40 receptor blockers: Rituximab (MabThera)

Immunomodulators

Alfetin

Cordyceps

Echinacea purpurea (However, here: http://www.rlsnet.ru/mnn_index_id_3204.htm it is written that the presence of autoimmune diseases is a contraindication for taking Echinacea purpurea)

Immunological tolerance is the ability of the immune system not to specifically respond to a particular antigen. For example, during pregnancy, the mother's immune system develops tolerance towards the embryo and placenta.

Violation of immune tolerance to self-antigens leads to the development of autoimmune diseases

Bruton's disease - (syn. - agammaglobulinemia, X-linked infantile, congenital agammaglobulinemia) is a variant of immunodeficiency. For the first time in 1952, the American pediatrician Bruton described an 8-year-old boy who suffered from various infectious diseases, who, from the age of 4, suffered from pneumonia 14 times, suffered from otitis media, sinusitis, sepsis, and meningitis. During the examination, no antibodies were found in the blood serum.

Mutant protein - Bruton's tyrosine kinase. The mutant BTK gene is mapped to Xq21.3-22.2.

Inheritance

An X-linked recessive type of inheritance is detected only in boys with the XY set of sex chromosomes. Girls do not get sick, because even if they are heterozygous, the recessive gene of one X chromosome is compensated by the normal gene of the homologous X chromosome.

Clinical manifestations

The age of onset of the disease is infancy or the 1st year of life; most often the disease manifests itself after 3-4 months of life. Patients suffer from recurrent infections caused by pneumococci, staphylococci and other pyogenic bacteria. Polio vaccination can be complicated by polio. Hepatitis B virus causes progressive, often fatal viral hepatitis. Infection with rotavirus or Giardia leads to chronic diarrhea and malabsorption syndrome. The lungs are primarily affected paranasal sinuses nose The clinical picture includes fever, malabsorption syndrome, conjunctivitis, central nervous system lesions (encephalitis), autoimmune diseases, and malignant neoplasms. Systemic rheumatic manifestations of the type of diffuse connective tissue diseases are possible. Joint syndrome characterized by episodic migratory polyarthralgia or arthritis large joints. Even with long term arthritis does not lead to radiological changes in the affected joints. There are skin lesions - eczema, dermatomyositis.

Laboratory diagnostics

A laboratory blood test reveals the absence of the gammaglobulin fraction in the proteinogram. Ig A and Ig M are reduced by 100 times, and the level of Ig G by 10 times. B lymphocytes are reduced. Plasmocytes in the bone marrow are reduced to the point of complete absence. Leukopenia or leukocytosis is observed in the peripheral blood.

The thymus is not changed, but the structure of the lymph nodes (in the biopsy specimen there is a narrowing of the cortical layer, the primary follicles in it are rare and underdeveloped) and the spleen are disturbed. X-ray reveals hypoplasia or absence lymphoid tissue(lymph nodes), hypoplasia or absence of pharyngeal lymphoid tissue (tonsils, adenoids).

Treatment - replacement therapyγ-globulin, plasma. The dose is selected so that the level of immunoglobulins in the blood serum is 3 g/l (first dose - 1.4 ml/kg, then 0.7 ml/kg every 4 weeks). Gamma globulin must be administered throughout life. During periods of exacerbation, antibiotics are used, most often semisynthetic penicillins and cephalosporins in normal dosages.

Immunochemistry studies chemical bases immunity. The main problems are the study of the structure and properties of immune proteins - antibodies, natural and synthetic antigens, as well as the identification of patterns of interaction between these main components of immunological reactions in different organisms.

Immunochemical methods are also used for applied purposes, in particular in the isolation and purification of the active principles of vaccines and serums.

Immunodeficiency conditions are divided into primary (congenital) and secondary. Among secondary immunodeficiency conditions, three forms are distinguished:

Purchased - most a shining example is acquired immunodeficiency syndrome - AIDS.

Induced - occurs as a result of specific reasons that caused its appearance: X-ray radiation, trauma and surgical interventions, the use of corticosteroids, cytostatic therapy, as well as immunity disorders that develop secondary to the underlying disease (diabetes, liver disease, kidney disease, malignant neoplasms) .

Spontaneous form - characterized by the absence of an obvious cause that caused a violation of immune reactivity.

The question of choosing a specific immunomodulatory drug and the need for its inclusion in the complex of therapy prescribed for the underlying disease should be decided by an immunologist, taking into account the clinical manifestations of immune deficiency and identified defects in the parameters of the immune status.

Immunodeficiency conditions (IDS) are a group of diseases based on immunopathology. Immunopathological conditions manifest themselves as the main clinical syndromes:

Infectious syndrome;

Immunoproliferative/oncological;

Allergic;

Autoimmune.

Recurrence of acute infections;

Protracted, sluggish nature of the disease;

A pronounced tendency to generalize the infectious process;

High risk of chronic disease, with frequent subsequent exacerbations and steadily progressive nature of the pathological process;

Early, rapid accession of opportunistic microflora;

The leading role of mixed infection in the formation of the inflammatory process;

Unusual pathogens;

Atypical forms of diseases;

Severe disease;

Opportunistic infections;

Resistance to standard therapy (combination of antibacterial agents, need for intravenous antibiotics, their long-term use and frequent change, lack of etiological recovery after repeated courses of treatment, expansion of drug prescriptions using immunotropic drugs, etc.).

Allergic syndrome is an immunopathological condition as the pathogenetic basis of the clinical manifestations of allergic diseases. Immunity disorders in the form of changes in the differentiation processes of immunoregulatory T-lymphocytes, hyperproduction of IgE, and decreased production of IgA determine the immune profile of patients with atopy, most likely due to genetic factors. Clinical manifestations of allergic syndrome are allergic diseases.

Lymphoproliferative/oncological syndrome is an immunopathological condition characterized by a decrease in the body's antitumor resistance and the development of cancer.

Autoimmune syndrome is an immunopathological condition associated with a violation of the mechanisms of autotolerance to antigens of one’s own body. Clinically manifests itself as autoimmune diseases or an autoimmune component during the inflammatory process.

IDS can manifest itself as isolated syndromes or their combinations.

There are two large groups immunodeficiencies - primary (congenital) and secondary (acquired). Primary IDS are congenital disorders of the immune system, characterized by early clinical manifestation of immunopathology. Most primary IDS are inherited conditions. The predominant type of inheritance is autosomal recessive, while many classic forms of primary IDS are inherited linked to the X chromosome, so up to 80% of the structure of primary IDS are boys. The clinical manifestation of primary IDS begins with an expansion of the antigenic load in the early childhood. At the same time clinical picture Primary immunodeficiency is determined by the level of damage to the immune system, i.e. specific syndrome and component factors: living conditions, state of local immunity, heredity, concomitant pathological conditions of other organs and systems, adaptive capabilities of the body, early diagnosis of immunodeficiency and therapeutic measures.

The classification of primary IDS uses the concept of syndrome. When naming a syndrome, a specific laboratory indicator is taken as a basis - a detectable defect, for example, hyper-IgM syndrome; a clear clinical sign, for example: ataxia telangiectasia; etiological factor, for example: Staphylococcus aureus syndrome; names of the authors who first described this syndrome or the name of the patient on whose example the syndrome was first described, for example: Wiskott-Aldrich syndrome, Jobe syndrome.

Classification of primary IDS (Stephanie D.V., Veltishchev Yu.E., 1996)

I. Insufficiency of the humoral immunity (B-lymphocyte system).

1. Agammaglobulinemia, Bruton's disease.

2. Dysgammaglobulinemia:

a) general variable hypogammaglobulinemia;

b) selective IgA deficiency;

c) deficiency of immunoglobulins IgG and IgA with increased synthesis of IgM - hyper IgM syndrome;

Immunodeficiency(ID) is a genetic and/or laboratory sign of a defect (insufficiency) of the immune system with or without clinical manifestations.

General signs of immunodeficiency disease:

The presence of an acute or recurrent (chronic) inflammatory infectious process of any localization. Viral and/or bacterial infections in newborns.

Detection of viruses, opportunistic bacteria and/or fungi in the lesion.

Clinical signs characteristic of primary immunodeficiencies in children.

The presence of causes (immunosuppressive factors) that caused acquired IBD.

Laboratory signs of immunodeficiency.

For diagnosis, the first two signs, in combination or without the 3rd and 4th, are sufficient.

Infectious syndromes of any localization are the main clinical “markers” of immunodeficiency and serve as clinical manifestations of immunodeficiency disease. The connection with infection “caused” by opportunistic microorganisms (viruses, bacteria, fungi) with immunodeficiency is obvious, because Only in its presence is their expansion possible – infection. It is the insufficiency of antiviral or antibacterial immunity that leads to the proliferation of these microorganisms - autologous or coming from outside.

The state of resistance and immunity of the body are the determining factors in the development of any infection.

As for opportunistic microorganisms - the vast majority of viruses, bacteria, fungi - the development of infection with their participation is possible only in an immunodeficient organism, i.e. if available absolute, and not a relative immunodeficiency of some factor, link, receptor or molecule of immunity.

Therefore, without immunodeficiency there is no infection, but there is one clinical manifestation IDB. Therefore, like infections, IBD have an acute, subacute and chronic course.

Distinguish primary And secondary immunodeficiencies (ID) and, accordingly, immunodeficiency diseases.

Primary IDs - This genetic abnormalities, usually clinically manifest (although not always!) in children. Secondary IDs occur in clinically healthy people under the influence of various reasons, however, in many of them a genetic predisposition to the development of IBD can be identified.

Primary combined immunodeficiencies

Severe combined ID (SCID) .

In this condition, the differentiation of various cells, including stem cells, is affected. There are several options for SCID.

Severe combined immunodeficiency with reticular dysgenesis. Mechanism: the differentiation and proliferation of hematopoietic stem cells into lymphoid and myeloid stem cells is impaired. Agranulocytosis and absence of lymphocytes are observed.

Children die in the first months of life from a septic process.

Severe immunodeficiency with reduced or normal numbers of B cells. Mechanism and clinic: defect in the gene responsible for the common γ-chain of cytokine receptors (IL-2, -4, -7) or the Jak 3 protein kinase gene; in the first 6 months of life, the child begins to have a persistent infection of the lungs, candidomycosis of the pharynx, esophagus, and diarrhea. There is a quantitative and/or functional deficiency of T cells, the content of B cells may correspond to the norm or exceed it, but these cells weakly secrete immunoglobulins, the levels of immunoglobulins A, M, G are reduced.

Immunodeficiency manifested by ataxia-telangiectasia (Louis-Bar syndrome).

Mechanism of ID: mutations, inversions and translocations in chromosomes 7 and 14, rearrangement of the T-receptor gene and other changes.

The clinical picture is polymorphic, changes in the immune system in the initial phase of the disease are insignificant or not observed; Neurological and vascular disorders, scleral and skin telangiectasia, cerebellar ataxia, ovarian dysgenesis may predominate; subsequently the damage to the immune system intensifies; the development of protracted, sluggish and chronic pneumonia is characteristic; death from infectious and neurovascular disorders.

The level of T-lymphocytes is reduced; levels of IgG, IgG2, IgG4 are observed, a response to FHA and bacterial antigens, disimmunoglobulinemia, and often there is a deficiency of IgA; Sometimes thymic hypoplasia and lymph node atrophy, Tx/Tc imbalance occur.

Wiskott-Aldrich syndrome.

Mechanism: the gene in Xp11 is defective was and therefore the expression of the glycosylated acidic glycoprotein, sialoporphyrin (CD43), involved in the activation of T cells, is impaired; autosomal recessive type of inheritance. Frequency – 4:1/million children.

Clinically manifested by a triad of symptoms - a combination of eczema, thrombocytopenia, and recurrent infection.

There is lymphocytopenia, T-lymphopenia, a decreased level of T-helper cells, thrombocytopenia, there are no PCT reactions determined by skin tests; the response of lymphocytes to PHA and antigens is reduced; significantly reduced IgM levels, high levels of IgA and IgE, normal or high level IgG, decreased production of antibodies to pneumococcal polysaccharides; macrophages do not break down polysaccharide antigens.

Clinic: thrombocytopenia at birth; bleeding; eczema; children in the first months of life experience repeated purulent infections caused by pneumococci and other polysaccharide-containing bacteria; splenomegaly; malignant tumors (5-12%); there is pronounced hypoplasia of the thymus gland and lymphoid tissue.

T cell immunodeficiencies

In these conditions, the T-link of the immune system is predominantly damaged.

Aplasia or hypoplasia of the thymus - DiGeorge syndrome.

Mechanism: broken embryonic development structures of the 3-4th pharyngeal pouches, deletion in chromosome 22q11, the epithelium of the thymus and parathyroid glands does not develop. There is a deficiency of T-cell function; the number of lymphocytes and their functional activity is reduced, the level of IgE is increased.

Clinic: aplasia or hypoplasia of the thymus; malformations: cleft palate, anomaly of the right aortic arch, underdevelopment of large vessels, sternum; cataracts, neonatal tetany due to underdevelopment of the parathyroid glands; frequent infectious complications; there are no PCZT reactions; the number of lymphocytes in the thymus-dependent areas of the lymph nodes is reduced.

Nezelof syndrome .

It is characterized by thymic hypoplasia, disruption of the normal maturation of T-lymphocytes, and their deficiency in the T-dependent zones of the immune system. The functions of T-cells are sharply suppressed, the total number of lymphocytes is reduced, the synthesis of immunoglobulins is normal or reduced, and antibody formation is suppressed.

Adenosine deaminase (ADA) deficiency.

Mechanism: genetic defect in the locus of the 20th chromosome – 20.q12 – 13.11, inherited in a recessive manner; there is a “silent” allele of the ADA locus; its deficiency in erythrocytes and lymphocytes leads to the accumulation of deoxyadenosine, which has a toxic effect on T-lymphocytes. Already in the first weeks of life, lymphocytopenia is noted; insufficiency of T-lymphocytes, appears immediately after the birth of a child, is combined with anomalies of skeletal development (deformation, ossification), signs of involution of the thymus gland are revealed.

B cell immunodeficiencies

With these deficiencies, the B-link of the immune system is predominantly damaged.

X-linked agammaglobulinemia with a growth hormone defect (Bruton's disease).

Boys get sick because due to a mutation in the Xq22 gene long shoulder X chromosome has no tyrosine kinase btk, structural genes for the synthesis of immunoglobulins do not function. Recessive type of inheritance linked to the X chromosome. Absent or sharply (less than 200 mg/l) reduced levels of IgM, IgG and IgA; there are no plasma cells in the lymphoid tissue and mucous membranes.

The clinical manifestations appear at 2–3 years of life: the body’s resistance to bacteria and fungi is reduced, and resistance to viruses is normal; there are no reactions of the lymph nodes, spleen during periods of exacerbation of the process, there is no enlargement of the adenoids, hyperplasia of the tonsils, combinations with atopic eczema, allergic rhinitis, bronchial asthma. Currently, with immunoglobulin replacement therapy, patients can live quite a long time.

Disimmunoglobulinemia .

This is a selective deficiency of one or more classes of immunoglobulins. The most common of these is selective immunoglobulin A deficiency (1:70-1:100). This defect may be asymptomatic, but relapses of respiratory and digestive diseases are often associated with it, because it protects the mucous membranes from microbes.

Selective IgM or IgG deficiencies are rare. Patients with IgM deficiency usually die from sepsis. IgG deficiency can present with a variety of symptoms depending on the IgG subclasses (usually IgG2) that are missing. Deficiency of immunoglobulins class E is not clinically manifested, however, there is a syndrome of IgE-hypergammaglobulinemia, which is characterized by various allergic manifestations, as well as chronic bacterial infections.

System defects mononuclear phagocytes and granulocytes

Based on their mechanism, such IDs can be divided into four groups.

The first group includes IDs associated with insufficient enzyme activity, resulting in digestive disorders absorbed object.

The second group includes IDs caused by a violation chemotaxis phagocytes.

The third group of ID is associated with insufficiency opsonizing factors blood serum (antibodies and complement).

The fourth group is characterized by insufficient receptor expression on the surface of macrophages (for the C3 component of complement, for Fc fragments of Ig, etc.).

For example, when deficiency of leukocyte adhesins (LAD-I syndrome) due to a gene defect, the CD18 molecule is missing, and they do not adhere to the endothelium and do not migrate into tissues.

Chronic granulomatous disease characterized by the fact that polynuclear cells are capable of phagocytosis, but do not digest absorbed microbes. This process is based on a defect in NADP oxidase, which catalyzes the conversion of oxygen into superoxide anion, which is necessary for the manifestation of the bactericidal activity of neutrophils. Catalase-positive staphylococci, Klebsiella, Salmonella, Escherichia coli, and fungi persist in phagocytes. At 1-4 years of age, children develop eczematous dermatitis, purulent skin lesions, abscesses in various organs, lymphadenitis, bronchopneumonia, and a fungal infection.

Laboratory diagnostic criteria are the absence of killing of phagocytosed bacteria, negative and reduced NBT test, chemiluminescence after phagocytosis of zymosan or latex particles.

Chediak–Higashi syndrome clinically characterized by increased sensitivity to purulent and viral infections and weakening of the color of hair, skin and iris of the eyes. Giant granules appear in the cytoplasm of neutrophils and macrophages, formed as a result of the fusion of cytoplasmic granules, which are revealed by staining for peroxidase. At the same time, pathological aggregation of melanosomes and, as a consequence, albinism are observed. The increased susceptibility to infection is explained by a disruption in the process of myeloperoxidase entering the vacuoles and their weak response to chemotactic stimuli.

Complement system deficiency

In the complement system, a deficiency of any component can be observed, and the absence of any factor blocks the activation of subsequent ones. This is accompanied by the development of various pathological conditions. Deficiency of C1, C2, C4 and C5 manifests itself in a syndrome similar to systemic lupus erythematosus. C3 deficiency is characterized by recurrent purulent infections.

In addition to the deficiency of the main components, there are deficiencies of complement system inhibitors: C1-inhibitor and C3-inactivator. Clinically, C1 inhibitor deficiency manifests itself hereditary angioedema . Edema of the larynx, extremities, and others occurs due to an increase in the concentration of a fragment of the C2 component, which has a vasoactive effect. Typically, such patients are heterozygous and synthesize a small amount of inhibitor. Its level can be increased by administering anabolic steroids or by performing replacement therapy with the inhibitor itself.

Directions for treatment of primary immunodeficiencies

Transplantation of bone marrow, neonatal thymus, fetal liver - in order to replace missing cells and create conditions for their full differentiation. Transplantation is used for severe combined ID.

Replacement therapy with immunoglobulins, enzymes, thymus hormones, mediators, vitamins and other factors.

Antibacterial therapy for concomitant infection.

Gene therapy: introduction of normal genes into SI cells (lymphocytes). The first to introduce the adenosine deaminase gene into the lymphocytes of patients with deficiency of this enzyme.

Secondary immunodeficiencies

Secondary (acquired) IDs are formed under the influence of the environment and are much more common than primary ones.

Signs of secondary ID:

absence of hereditary condition

occurs against the background of normal body reactivity

connection with causative factor, which determined the ID

Causes of secondary ID

1. Environmental adverse effects on the body and immune system (physical, chemical, biological).

2. Diseases that affect the immune system:

– viral (more often)

– allergic and autoallergic, oncological

– metabolic disorders, cell proliferation, protein loss

– other serious illnesses

3. Immunosuppressive treatments:

– drug immunosuppression

– radiation and other types of energy in large doses

– surgical interventions and anesthesia

– graft-versus-host disease (GVHD) after bone marrow allotransplantation

4. Physical and emotional stress

5. Insufficient nutrition and exhaustion (protein, fat-carbohydrate, vitamin, microelement deficiency).

6. Occupational harmful factors (chemical, physical, psycho-emotional).

7. Age: prematurity of children and the pathology of aging (“elderly syndrome”)

The secondary, like the primary, ID can be hidden and not have clinical signs and is detected only during laboratory examination. ID with clinical signs is immunodeficiency disease . Clinically, it manifests itself as chronic purulent-inflammatory infections of the skin, upper respiratory tract, lungs, genitourinary system, gastrointestinal tract and other organs. They differ from transient changes in the immune system by the persistence of disturbances in the immune system after the end of the causative factor.

By severity clinical course differentiate lungs, moderate, subcompensated and severe decompensated secondary immunodeficiency diseases (IDDs) .

Viral secondary immunodeficiency diseases

Viruses often persist in the human body without manifestations of pathology, i.e. Widespread virus carriage is noted. This applies to herpes viruses, cytomegalovirus, adenoviruses, Epstein-Barr virus and many others. With a decrease in the level and deficiencies in the interferon system, they are able to induce immunodeficiency and, therefore, IBD in several ways:

– transforming the genome of SI cells;

– directly destroying immunocompetent cells,

– inducing apoptosis;

– binding to receptors and changing their activity, chemotaxis, activating suppressors;

– binding or releasing cytokines, i.e. modifying immunoreactivity.

Some viruses have the ability to replicate within immunocompetent cells themselves. An example of such a mechanism could be the known tropism for B lymphocytes of the Epstein-Barr virus or the selective defeat of T helper cells by the HIV virus. Viruses of many acute infections, in particular measles, influenza, rubella, chickenpox, mumps, herpes - can cause transient anergy to other antigens. Clinically, transient immunosuppression is expressed in the development of viral and bacterial complications, often observed in these infections. Persistence of hepatitis viruses can lead to immunomodulation and T-cell suppression.

Transplacentally transmitted viruses (cytomegaly, rubella) have a damaging effect on various tissues, including SI cells. The most significant defects have been described in congenital rubella and cytomegaly. Some children showed a lack of humoral and cellular immune response to antigens, while others had a selective IgA deficiency. The latter defect is explained by the ability of the virus to block the development of cells at the intermediate stage of differentiation.

Immunosuppression during active cytomegalovirus infection in children is manifested by a decrease in the number of CD3 +, CD4 + T-lymphocytes, and inhibition of the phagocytic activity of neutrophils. Such children are predisposed to the development of bacterial and viral superinfections.

Disturbances in the composition of T- and B-lymphocytes are observed during herpes infection, when the number of T-activated lymphocytes increases against the background of general T- and B-lymphocytopenia and a decrease in the expression of HLA system molecules. Chronic persistence of the herpes virus in leukocytes and nerve ganglia leads to the development of ID.

There is a complex pathogenetic relationship between viral infection and SI deficiency. On the one hand, a viral infection can induce secondary immunodeficiency; on the other hand, in patients with immunodeficiency, viral superinfection causes severe, life-threatening conditions, i.e. strengthens this ID.

Persistent viruses and intracellular immunity

Many viruses - herpes, cytomegalovirus (CMV), Epstein-Barr, rhinoviruses, enteroviruses - are constantly present in the cells of the body and, periodically activated, induce various clinical manifestations. A striking example is herpes viruses of types 1-8, which persist in the nerve ganglia and cause lesions of the skin and mucous membranes according to the level of localization of the ganglia - labial, cockroach (herpes zoster), sacral (genital). Herpes viruses type 8 persist in T-lymphocytes, Epstein-Barr - in B-cells and others, CMV - in macrophages, leukocytes, and epithelial cells. In most people who carry them, they do not cause infections, which is apparently due to fairly high immunity, primarily interferon, because they do not replicate.

A striking example of virus-induced ID is HIV infection. The human immunodeficiency virus (HIV) causes an infectious disease associated with primary lesions of the SI and the development of severe secondary immunodeficiency, against the background of which opportunistic and non-pathogenic microflora are activated.

Secondary immunodeficiencies in diseases

All serious diseases lead to the development of immunological deficiency.

One of the causes of secondary immunodeficiency is metabolic disorders. In diabetes mellitus, for example, the chemotaxis and phagocytic activity of neutrophils are inhibited, resulting in skin pyoderma and abscesses.

For burns ID occurs due to the large loss of immunoglobulins and complement proteins in the blood plasma. If the area of skin damage exceeds 30%, cellular immunity disorders develop.

Tumors secrete immunomodulatory factors and cytokines that suppress immunity. There is a decrease in the number of T-lymphocytes, an increase in the activity of suppressor cells, and inhibition of phagocytosis. Particularly pronounced changes occur in widespread tumor processes with metastasis.

Secondary immunodeficiencies in various pathophysiological conditions and stress

In chronic starvation, immunodeficiencies arise due to a lack of proteins, vitamins and microelements. In these cases, the cellular immune system suffers first of all: the response of lymphocytes to mitogens decreases, atrophy of lymphoid tissue is observed, and the function of neutrophils is impaired.

Heavy physical activity and concomitant stress in professional athletes, depending on the duration of the load, cause temporary or persistent immunomodulation. There is a decrease in the level of immunoglobulins, subpopulations of T-lymphocytes, and phagocytosis activity. During this “immunodeficiency period,” athletes are highly susceptible to various infections. SI values usually normalize during the rest period, but not always.

Secondary IDs at surgical operations are associated with a powerful stress response and with the effect of drugs for anesthesia. A temporary immunodeficiency state develops, in which the number of T- and B-lymphocytes decreases and their functional activity decreases. Impaired indicators are restored only after a month, if there are no factors that suppress immunity.

When aging In the body, IDs are the result of immunomodulations arising from exposure to unfavorable factors and diseases, especially viral ones. In healthy elderly people (90-100 years old), SI indicators are close to their values in middle-aged people, although they have their own characteristics.

Newborns and children early age have SI indicators different from those of adults; they have maternal IgG circulating, obtained through the placenta, the level of which decreases at 3-6 months, which is not ID. Premature babies are born with various SI defects associated both with its immaturity and often with intrauterine infections. Artificial feeding of children causes deficiency of secretory IgA and other protective factors(lysozyme, etc.) of mother's milk.