Immunodeficiency symptoms. Secondary immunodeficiencies. Causes and symptoms of immunodeficiency

is a condition in which there is a decrease in work performance immune system and the body's resistance to various infections.

Immunodeficiencies are divided into:

Primary immunodeficiencies

A group of diseases characterized by decreased functioning of the immune system resulting from genetic disorders. Primary immunodeficiencies are very rare (one to two cases per 500,000). With primary immunodeficiency, there is a violation of individual components of immunity: the complement system and phagocytes, the humoral response, the cellular link.

Immunodeficiency with a violation of the cellular component of the immune system includes agamaglobulinemia, Wiskott-Aldrich syndrome, DiGiorgio syndrome, Bruton's disease. Failure of the function of micro and macrophages occurs during the period of Chediak-Higashi syndrome and chronic granulomatosis.

Immunodeficiencies that are associated with a malfunction of the compliment system are based on a lack of synthesis of one of the factors of this system. Primary immunodeficiencies accompany a person throughout life. People suffering from primary immunodeficiency often die from infectious complications.

Secondary immunodeficiencies

These immunodeficiencies are more common than primary ones. As a rule, the development of secondary immunodeficiency occurs as a result of exposure to unfavorable factors environment and various infectious diseases.

With secondary immunodeficiency, just as in the case of primary immunodeficiency, either individual components of the immune system or the entire system may be impaired. Many secondary immunodeficiencies are treatable. However, this does not apply to immunodeficiency, which is caused by HIV infection.

Causes of secondary immunodeficiency.

Factors that can cause secondary immunodeficiency are quite diverse. This can be caused by environmental factors or internal factors of the body. Unfavorable environmental factors can disrupt the metabolism of the entire body, or cause secondary deficiency.

The most common causes of immunodeficiency are poisoning, long-term use of certain medicines, microwave and ionizing radiation, overwork, chronic stress and environmental pollution.

Internal factors that can cause secondary immunodeficiency:

Malignant tumors (neoplasms) that disrupt all body systems. A more obvious decrease in immunity is manifested in the replacement of bone marrow by tumor metastases and in malignant blood diseases (leukemia). Against the background of leukemia, the number of immune cells in the blood increases many times. However, they cannot provide a protective function, as they are non-functional.

Autoimmune diseases that form as a result of a malfunction of the immune system. Due to illness of this type The immune system begins to work insufficiently, which leads to damage to its own tissues and a lack of ability to fight infection.

Malnutrition, exhaustion of the body, which lead to decreased immunity. As a result of exhaustion of the body, malfunction occurs internal organs. The immune system is especially sensitive to vitamin deficiency, nutrients and minerals. A decrease in immunity often occurs during periods of vitamin deficiency - in winter and spring.

Loss of immune defense factors, which is observed with burns, kidney disease and severe blood loss. A feature of these diseases is the loss of blood plasma or the dissolution of proteins in it, some of which are immunoglobulins or other components of the immune system (C-reactive protein or complement system proteins). During the period of bleeding, loss of plasma and blood cells occurs, which leads to a decrease in immunity, which is cellular-humoral in nature.

Endocrine diseases that lead to decreased function of the immune system as a result of metabolic failure. A more intense decrease occurs in hypothyroidism and diabetes mellitus, since in these diseases the production of tissue energy is significantly reduced, which entails a failure in cell differentiation and division processes. Diabetes mellitus increases the incidence of infectious diseases, which is associated with suppression of the immune system and high content glucose in the blood, which promotes the growth of bacteria.

Serious operations and injuries that occur with a decrease in the performance of the immune system. Any serious illness can lead to secondary immunodeficiency, which may be associated with intoxication of the body, metabolic disorders, secretion large quantity hormones by the adrenal glands after operations or injuries that can lead to suppression of the immune system.

Taking drugs and medicines, which have an intense immunosuppressive effect. This is especially evident after taking antimetabolites, glucocorticoid hormones and cytostatics.

Decreased immunity in older people, children and pregnant women, which is associated with physiological or age-related characteristics of the body.

Diagnosis of immunodeficiency.

Primary immunodeficiency appears at birth or after some time. To identify pathology, a series of complex genetic and immunological tests are carried out, which can determine the area of ​​​​the immune system disorder and determine the type of mutation that caused the disease.

Secondary immunodeficiency develops at any age. The presence of secondary immunodeficiency can be suspected in case of recurrent infectious diseases, which can become chronic, in the absence of treatment results and with a prolonged slight increase in body temperature.

To ensure accurate diagnosis, tests and analyzes are carried out: specific immunological tests, determination of blood protein fractions, general blood test.

Treatment of primary immunodeficiency.

A difficult task is the treatment of primary immunodeficiencies. To get started complex treatment it is necessary to determine the accuracy of the diagnosis of the disease with the identification of the damaged part of the immune system.

If the amount of immunoglobulin is insufficient, lifelong replacement therapy is carried out using sera that contain regular donor plasma or antibodies.

Immunostimulating therapy is used with drugs such as Taktivin, Ribomunil, Bronchomunal. If infectious complications develop, treatment with antifungals is prescribed, antiviral drugs and antibiotics.

Treatment of secondary immunodeficiency.

With secondary immunodeficiency, the failure of the immune system does not occur as intensely as with primary immunodeficiency. Secondary immunodeficiency has a transient nature, which contributes to greater effectiveness of treatment.

As a rule, treatment begins with eliminating the causes that contributed to the development of the disease. For example, treatment of immunodeficiency, which arises as a result of chronic infection, begins with the sanitation of foci of inflammation.

Immunodeficiency, which was caused by vitamin and mineral deficiency, is treated with nutritional supplements, minerals and vitamins. The restorative ability of the immune system is so high that eliminating the cause of immunodeficiency leads to the restoration of immunity.

In order to speed up recovery, you can undergo a course of treatment with drugs that promote immunostimulation.

Drugs such as Biostim, Christine and Ribomunil contain antigens of various bacteria and, when introduced into the body, stimulate the differentiation of leukocyte clones and the production of antibodies. Taktivin and Timalin contain biological active substances that are extracted from the thymus gland of animals. The most effective immunomodulator is Cordyceps, which helps normalize the immune system in general.

These drugs promote a selective stimulating effect on a subpopulation of T lymphocytes. Varieties of interferons can increase the body's resistance and are used in the form effective remedy for the treatment of viral diseases. Sodium Nucleinate is used to stimulate the synthesis of nucleic acids (RNA and DNA), differentiation and cell division.

Special attention You should pay attention to immunomodulatory substances of plant origin: rosea Echinacea extract, Immunal and especially Cordyceps.

Secondary immunodeficiency- symptoms and treatment

What is secondary immunodeficiency? We will discuss the causes, diagnosis and treatment methods in the article by Dr. E. Yu. Bychkova, an immunologist with 21 years of experience.

Publication date September 19, 2019Updated October 04, 2019

Definition of disease. Causes of the disease

Secondary immunodeficiency is a pathological condition of the body in which a defect in one or more immune response mechanisms leads to a disruption of the immune system as a whole. It develops against the background of the underlying disease.

Secondary immunodeficiency develops in people over 16 years of age with an initially normally functioning immune system. Usually its appearance is associated with exposure to the environment or some other factors, but in many of the patients a genetic predisposition to the formation of immune problems can be identified.

Immunologically healthy body able to control and prevent the development of infections and tumor processes, that is, to exercise immunological control of the internal environment. With the development of secondary immunodeficiency, the main “marker” of the problem becomes the development of infections caused by opportunistic agents (viruses, bacteria and fungi), and the activation of infections that usually do not occur in people with healthy immunity. Therefore, the main signs of secondary immunodeficiency are relapses and exacerbations of infections - acute, subacute and chronic.

Reasons for the development of secondary immunodeficiency states:

If you notice similar symptoms, consult your doctor. Do not self-medicate - it is dangerous for your health!

Symptoms of secondary immunodeficiency

Clinical manifestations of secondary immunodeficiency are varied. Very often, the symptoms of the underlying disease that led to the formation of immunodeficiency come to the fore. But the main manifestations of problems with immunity, which indicate the need for an immunological examination, are still present. These include:

• frequent colds - more than six times a year in children and more than four times a year in adults;
• the duration of each episode of the infectious-inflammatory process is more than two weeks;
• recurrence of infections that usually do not occur in people with healthy immune systems (herpes, infections caused by Candida fungi);
• recurrence of bacterial infections (furunculosis, hidradenitis, gynecological and ENT diseases);
• a slight increase in temperature of unknown origin, that is, such possible reasons, like tuberculosis and cancer;
• recurrence of lymphadenitis and lymphadenopathy (inflammation of the lymph nodes);
• asthenic syndrome - weakness, fatigue, decreased performance, feeling exhausted after 8 hours of sleep;
• chronic ulcers and non-healing wounds.

All chronic infections and inflammation occurs against the background of weakened immune defense. Therefore, any chronic infection will indicate a failure of the immune system, which cannot cope with the disease during an acute inflammatory process.

It should be remembered that best option immune protection for acute infection should end with recovery on average within 2-4 weeks from the onset of the disease.

Pathogenesis of secondary immunodeficiency

During life, the body is exposed to various damaging factors that cause secondary immunodeficiency. They lead to inhibition of cellular immune responses, a decrease in leukocytes and interferons - proteins of the immune system that are produced by leukocytes and immune cells and block the spread of the virus. As a result, chronic infectious and inflammatory processes appear, often relapsing.

Some trace elements can regulate the functioning of the immune system, for example, zinc, iodine, lithium, copper, cobalt, chromium, molybdenum, selenium, manganese and iron. Their lack aggravates immune dysfunction.

Lack of vitamins, imbalance of macro- and microelements caused by an unbalanced diet lead to disruption of the cellular immune system: the response of lymphocytes to mitogens (substances that stimulate T cells) is reduced, atrophy of lymphoid tissue is observed, the function of neutrophils (blood cells that suppress harmful substances) is impaired ) .

The mechanism of development of secondary immunodeficiency can be most clearly demonstrated using the example of viruses of the herpes family. Many viruses (herpes simplex viruses of the first and second types, cytomegalovirus, Epstein-Barr virus, rhinoviruses, enteroviruses) are constantly present in the cells of the body. Periodically activated under the influence of stress, unbalanced nutrition, concomitant pathology or immunosuppressive therapy, they contribute to the appearance of various clinical manifestations.

Tumors that arise in the body with weakened immune defenses deplete the immune system as they grow, and the treatment (surgery, radiation and chemotherapy) also aggravates the resulting immunodeficiency. In this case, the cellular component of immunity suffers:

All serious diseases also lead to the development of immune deficiency. For example, in diabetes mellitus, chemotaxis (the movement of microorganisms in response to Chemical substance) and phagocytic activity of neutrophils (the number of cells involved in the capture of viruses and bacteria), protection against bacterial infections is impaired, as a result of which cutaneous pyoderma develops ( purulent diseases) and abscesses.

The body reacts to stress by releasing active substances, like catecholamines and glucocorticoids, which have an important modulating effect on the functioning of cells of the immune system. Under their influence, pro-inflammatory cytokines are released, processes are launched aimed at combating a possible infectious agent. With chronic stress, due to the prolonged influence of glucocorticoids on cells of the immune system, the level of immunoglobulins and the activity of phagocytosis decreases. As a result, the process of apoptosis and cell damage is triggered during oxidation - programmed death in cells of the immune system. If there was no excess load and the body had enough rest, then the functioning of the immune system returns to normal.

Classification and stages of development of secondary immunodeficiency

The form of secondary immunodeficiency can be:

There are two types of secondary immunodeficiency based on duration:

Based on a study of the immune status, secondary immunodeficiency is distinguished by the location of the main defect:

• 1. combined deficiency - changes affect several parts of the immune defense;
• 2. T-cell deficiency;
• 3. predominantly B-cell deficiency;
• 4. defect of natural killer cells;
• 5. deficiency of macrophages and granulocytes;
• 6. deficiency of the complement system;
• 7. platelet system deficiency;
• 8. deficiency of the interferon system.

It is almost impossible to establish the localization of damage to the immune system, based only on symptoms, without laboratory testing of immune defense indicators, since a similar clinical picture can be observed with different types of secondary deficiency. For example, a person may be prone to viral infections both with T-cell deficiency and with a deficiency of the interferon system.

Separately, it should be said about physiological immunodeficiency in pregnant women. It is reversibly formed in a woman’s body for the growth and development of the fetus and does not require immunocorrection.

Complications of secondary immunodeficiency

Secondary immunodeficiency developing against the background of a primary infectious and/or inflammatory disease, causes severe life-threatening conditions. The lack of timely correction of this state of immunity closes the pathological circle and worsens the course of the underlying disease.

The most common complications of secondary immunodeficiency are severe infectious diseases: sepsis, abscess and phlegmon. Their main manifestations are high fever and signs of inflammation. With pneumonia, shortness of breath, cough, pain in the chest. Sepsis develops when an infection enters the bloodstream and is accompanied by a systemic inflammatory response, multiple organ failure and is life-threatening condition. With abscesses and phlegmon, there is a focus in the body purulent inflammation with severe pain, swelling, hyperemia (redness).

Taking into account the great heterogeneity of these diseases, prognosis and possible complications should be determined individually, based on the course and severity of the underlying disease against which secondary immunodeficiency arose.

Diagnosis of secondary immunodeficiency

The diagnosis of secondary immunodeficiency is made based on history, physical examination, and testing of immune system function.

For appointment effective measures In order to correct immunity, it is very important to identify the factors that contributed to the development of immunodeficiency. Therefore, for a consultation with an immunologist, the patient should bring all the examinations he has, and the doctor will ask in great detail about the diseases suffered, the treatment performed, operations and the patient’s lifestyle.

If secondary immunodeficiency is suspected, the doctor prescribes a blood test and an immunogram - immunological tests that evaluate the quantitative and qualitative characteristics of the components of the immune system.

The immunogram consists of several blocks:

Secondary immunodeficiency may be indicated by both decreased and increased performance immunograms.

Since the immune system works according to a system of modules, in the early stages, a violation of immunity may be accompanied, for example, by an increase in T-link indicators or a deficiency of interferon status indicators. This picture is observed at the beginning chronic course herpetic infections. At later stages of secondary immunodeficiency, a decrease in indicators can already affect both links, which can often be seen in the immunograms of patients with common cancers.

If an imbalance of key nutrients (vitamins, microelements, etc.) is suspected, an immunologist may prescribe tests:

In his conclusion, the immunologist can diagnose “secondary immunodeficiency” as either the main diagnosis or a concomitant one. It all depends on the clinical picture.

Treatment of secondary immunodeficiency

Treatment of a patient with secondary immunodeficiency is carried out together with a doctor who supervises the patient regarding the underlying disease. The task of the immunologist is to select immunocorrective therapy. “Immune correction” in this case means treatment aimed at restoring the body’s weakened immune defense, correcting the imbalance of its components and immune reactions, weakening pathological immune processes and suppressing autoimmune reactions.

Treatment of secondary immunodeficiency begins with identifying and eliminating the cause of its occurrence. For example, in case of a disorder of immunity caused by an infectious-inflammatory process, sanitation (cleansing) of foci of chronic inflammation is required.

If secondary immunodeficiency occurs as a result of vitamin and mineral deficiency, then complexes containing deficient components are prescribed. For example, with the aging of the thymus gland and depletion of the lymph nodes, vitamins B6 are prescribed. During exacerbation or onset of autoimmune and lymphoproliferative diseases, vitamins E are indicated.

Important minerals for the immune system are zinc, iodine, lithium, copper, cobalt, chromium, molybdenum, selenium, manganese, iron. These trace elements are necessary for the activation of enzymes involved in immune reactions. For example, zinc prevents the death of immune system cells.

Sometimes the immune system cannot cope with the pathological process due to a deficiency of any protective factors (cells and cytokines). In such cases, the immunologist prescribes immunostimulating drugs to speed up recovery and stimulate the immune system.

Taking immunomodulators for acute infectious inflammation allows you to:

• reduce the depth of damage in inflamed tissues;
• reduce the time of rehabilitation and recovery;
• reduce the risk of fatal complications in severe cases;
• prevent chronic disease and the formation of resistant strains of infectious agents.

In chronic infectious inflammation, immunomodulators contribute to:

• a significant reduction in symptoms of the disease or complete recovery;
• a significant increase in the period of remission between exacerbations of recurrent infection.

The degree of effectiveness of immunocorrection is influenced by the frequency of its use and the stage of the disease. The duration of action of immunomodulators depends on the nature of the drug, indicators of immune status and the type of disease that caused secondary immunodeficiency.

Elimination of a deficiency in one part of the immune system can lead to compensation of another part, since the immune system operates on the principle of interconnected modules.

Either one immunomodulator or several, that is, a combination of them, can be prescribed. Under combined immunocorrection understand the sequential or simultaneous use of several modulators with by different mechanisms actions. Today it is known big number immunomodulatory drugs with different mechanisms of action.

Indications for combination immunotherapy are:

• chronicity of the main pathological process(when the illness lasts more than three months);
• frequent relapses of the underlying disease (more than four times a year);
• the presence of complications of the main pathological process;
• severe intoxication syndrome;
• impaired metabolism;
• unsuccessful immunocorrection with one drug for a month;
• damage to several links (phagocytosis, T- and B-links of immunity);
• the need for multidirectional effects on the links of immunity - stimulation of one link and inhibition of another.

IMPORTANT: You cannot prescribe immunocorrectors on your own, since their uncontrolled use can only provoke the development of secondary immunodeficiency.

Forecast. Prevention

In secondary immunodeficiency, immune system disorders are less pronounced than in primary immunodeficiency. In most cases, several factors are identified that suppress the immune response.

As a rule, the prognosis of secondary immunodeficiency is determined by the severity of the disease that caused it. For example, immunodeficiency caused by a lack of vitamins or a violation of the work-rest regime is much easier to compensate for than immunodeficiency caused by a tumor process, diabetes mellitus or HIV infection.

U child's body individual parts of the immune system are still immature. T-lymphocytes are just beginning to “get acquainted” with various infectious agents, so the child often suffers from various acute respiratory viral infections. Over time, “memory cells” accumulate. As people grow older, a wide “repertoire” of T-lymphocytes develops that can recognize and quickly trigger an immune response, and therefore the incidence of disease decreases.

As the body ages, so does the immune system. For example, due to age-related changes Fewer T cells are involved in the response to new antigens, cooperation between cells of the immune system worsens, and the efficiency of phagocytosis (absorption of viruses and dead cells) decreases. Therefore, many infectious and inflammatory diseases become much more severe with age and cause complications more often.

Primary prevention of secondary immunodeficiency is to maintain a healthy lifestyle, balanced diet, quitting smoking. All this helps prevent the development of various diseases.

Secondary prevention can be carried out with timely treatment infectious and general somatic diseases.

People with secondary immunodeficiency need " special conditions", which will prevent the worsening of immune disorders.

– a group of pathological conditions of a predominantly congenital nature, in which there is a disruption in the functioning of certain parts of the immune system. Symptoms vary depending on the type of disease, but generally involve increased susceptibility to bacterial and viral agents. Diagnosis of pathology is made through laboratory methods research, molecular genetic analysis (for hereditary forms), studying the patient’s medical history. Treatment includes replacement therapy, bone marrow transplantation, and infection control measures. Some forms of immunodeficiency are incurable.

General information

Primary immunodeficiencies have been actively studied since the 50s of the 20th century - after the first condition of this type, which received his name, was described in 1952 by the American pediatrician Ogden Bruton. Currently, over 25 types of pathology are known, most of of which are genetically determined diseases. The incidence of different types of immunodeficiency ranges from 1:1,000 to 1:5,000,000. The vast majority of patients are children under the age of 5 years, mild forms can be first detected in adults. IN in some cases an immunodeficiency state is detected only by the results of laboratory tests. Some types of the disease are combined with numerous malformations and have a high mortality rate.

Causes of primary immunodeficiencies

Primary immunodeficiency states begin to form at the stage intrauterine development under the influence of various factors. They are often combined with other defects (dystrophies, abnormalities of tissues and organs, fermentopathy). Based on etiology, there are three main groups: congenital pathologies immune system:

• Due to genetic mutations. The vast majority of diseases arise from defects in the genes responsible for the development and differentiation of immunocompetent cells. Autosomal recessive or sex-linked inheritance is usually observed. There is a small proportion of spontaneous and germline mutations.
• As a result of teratogenic effects. Congenital problems with immunity can result from exposure to toxins of various natures on the fetus. Immunodeficiency often accompanies developmental defects caused by TORCH infections.
• Unclear etiology. This group includes cases when it is not possible to identify the cause of the weakness of the immune system. These may be yet unexplored genetic abnormalities, weak or unknown teratogenic effects.

The study of the causes, pathogenesis and search for methods for treating primary immunodeficiencies continues. There are already indications of a whole group of similar conditions that do not manifest themselves severe symptoms, but under certain conditions can provoke infectious complications.

Pathogenesis

The mechanism of development of immune deficiency depends on the etiological factor. In the most common genetic variant of the pathology, due to the mutation of some genes, the proteins they encode are either not synthesized or have a defect. Depending on the functions of the protein, the processes of lymphocyte formation, their transformation (into T- or B-cells, plasma cells, natural killer cells) or the release of antibodies and cytokines are disrupted. Some forms of the disease are characterized by a decrease in the activity of macrophages or a complex failure of multiple components of the immune system. Varieties of immunodeficiency caused by the influence of teratogenic factors most often arise due to damage to the rudiments immune organs– thymus, bone marrow, lymphoid tissue. Underdevelopment of individual elements of the immune system leads to its imbalance, which is manifested by a weakening of the body's defenses. Primary immunodeficiency of any origin causes the development of frequent fungal, bacterial or viral infections.

Classification

The number of types of primary immunodeficiencies is quite large. This is explained by the complexity of the immune system and the close integration of its individual links, as a result of which disruption or “switching off” one part contributes to the weakening of the entire defense of the body as a whole. To date, a complex branched classification of such conditions has been developed. It consists of five main groups of immunodeficiencies, each of which includes several of the most common types of pathology. In a simplified version, this classification can be presented as follows:

1. Primary deficiencies of cellular immunity. The group includes conditions caused by insufficient activity or low levels of T lymphocytes. The cause may be thymus failure, fermentopathy and other (mainly genetic) disorders. The most common forms of immunodeficiency of this type are DiGeorge and Duncan syndromes, orotaciduria, and lymphocyte enzyme deficiency.
2. Primary deficits of humoral immunity. A group of conditions in which the function of predominantly B-lymphocytes is reduced and the synthesis of immunoglobulins is impaired. Most forms fall into the category of dysgammaglobulinemia. The most famous syndromes are Bruton, West, IgM or transcobalamin II deficiencies.
3. Combined primary immunodeficiencies. An extensive group of diseases with reduced activity of both cellular and humoral immunity. According to some data, this type includes more than half of all types of immune deficiency. Among them there are severe (Glanzmann-Riniker syndrome), moderate (Louis-Bar disease, autoimmune lymphoproliferative syndrome) and minor immunodeficiencies.
4. Primary phagocyte deficiency. Genetic pathologies that cause decreased activity of macro- and microphages - monocytes and granulocytes. All diseases of this type are divided into two large groups– neutropenia and defects in the activity and chemotaxis of leukocytes. Examples include Kostman neutropenia and lazy white blood cell syndrome.
5. Deficiencies of complement proteins. A group of immunodeficiency conditions, the development of which is caused by mutations in genes encoding complement components. As a result, the formation of the membrane attack complex is disrupted, and other functions in which these proteins participate suffer. It causes complement-dependent primary immunodeficiencies, autoimmune conditions or.

Symptoms of primary immunodeficiencies

Clinical picture various forms Immunity deficiency is very diverse and can include not only immunological disorders, but also developmental defects, tumor processes, and dermatological problems. This allows pediatricians or immunologists to differentiate different types of pathology even at the stage of physical examination and basic laboratory tests. However, there are certain general symptoms, similar in diseases of each group. Their presence indicates which link or part of the immune system was affected to a greater extent.

In primary deficiencies of cellular immunity, viral and fungal diseases. These include frequent colds, more severe than normal progression of childhood viral infections (chicken pox, mumps), and severe herpetic lesions. Candidiasis of the oral cavity and genital organs often occurs, and there is a high probability of fungal infections of the lungs and gastrointestinal tract. Individuals with deficiencies in the cellular component of the immune system have an increased risk of developing malignant neoplasms– lymphomas, cancer of various localizations.

The weakening of the body's humoral defense usually manifests itself hypersensitivity to bacterial agents. Patients develop pneumonia, pustular skin lesions (pyoderma), often becoming severe (staphylo- or streptoderma, erysipelas). When the level of secretory IgA decreases, the mucous membranes (conjunctiva of the eyes, surfaces of the oral and nasal cavities), as well as the bronchi and intestines are mainly affected. Combined immunodeficiencies are accompanied by both viral and bacterial complications. Often, it is not the manifestations of a lack of immunity that come to the fore, but other, more specific symptoms - megaloblastic anemia, malformations, tumors of the thymus and lymphoid tissue.

Congenital neutropenia and weakened phagocytosis of granulocytes are also characterized by frequent occurrence bacterial infections. Purulent-inflammatory processes with the formation of abscesses in various organs are not uncommon; in the absence of treatment, the formation of phlegmon and sepsis is possible. The clinical picture of complement-associated immunodeficiencies is presented either as a decrease in the body’s resistance to bacteria, or in the form of autoimmune lesions. A separate variant of complement-dependent immune disorder - hereditary ANO - is manifested by recurrent edema of various parts of the body.

Complications

All types of primary immunodeficiency have in common an increased risk of severe infectious complications. Due to the weakening of the body's defenses, pathogenic microbes cause severe damage to various organs. Most often the lungs (pneumonia, bronchitis, bronchiectasis), mucous membranes, skin, and organs of the gastrointestinal tract are affected. In severe cases of the disease, it is the infection that causes death in infancy. Concomitant disorders such as megaloblastic anemia, abnormal development of the heart and blood vessels, damage to the spleen and liver can lead to aggravation of the pathology. Some forms of immunodeficiency conditions in the long term can cause the formation of malignant tumors.

Diagnostics

In immunology, a huge number of techniques are used to determine the presence and identification of the type of primary immunodeficiency. More often, immunodeficiency conditions are congenital, so they can be detected already in the first weeks and months of a child’s life. Frequent bacterial or viral diseases, burdened hereditary history, the presence of other developmental defects. Varieties of mildly manifested immunodeficiencies can be determined later and are often discovered by chance during laboratory tests. The main methods for diagnosing hereditary and congenital immune disorders are:

• General inspection. You can suspect the presence of severe immunodeficiency even during examination skin. In sick children, severe dermatomycosis, pustular lesions, atrophy and erosion of the mucous membranes are often detected. Some forms also manifest as swelling of the subcutaneous fat.
• Lab tests. The leukocyte formula in the general blood test is disturbed - leukopenia, neutropenia, agranulocytosis and other anomalies are noted. With some varieties, an increase in the level of certain classes of leukocytes is possible. Biochemical analysis blood in case of primary immunodeficiency of the humoral type confirms dysgammaglobulinemia, the presence of unusual metabolites (in enzymopathies).
• Specific immunological studies. To clarify the diagnosis, a number of techniques are used aimed at determining the activity of the immune system. These include analysis of activated leukocytes, phagocytic activity of granulocytes, level of immunoglobulins (in general and individual fractions - IgA, E, G, M). They also conduct a study of the level of complement fractions, interleukin and interferon statuses of the patient.
• Molecular genetic analysis. Hereditary types of primary immunodeficiencies can be diagnosed by sequencing genes whose mutations lead to one form or another of the disease. This is how the diagnosis is confirmed for DiGeorge, Bruton, Duncan, Wiskott-Aldrich syndromes and a number of other immunodeficiency conditions.

Differential diagnosis is primarily carried out with acquired secondary immunodeficiencies, which can be caused by radioactive contamination, poisoning with cytotoxic substances, autoimmune and oncological pathologies. It can be especially difficult to discern the cause of the deficiency in smoothed forms, which are determined mainly in adults.

Treatment of primary immunodeficiencies

There are no uniform treatment principles for all forms of pathology due to differences in etiology and pathogenesis. In the most severe cases (Glanzmann-Riniker syndrome, Kostman agranulocytosis), any therapeutic measures are temporary, and patients die due to infectious complications. Some types of primary immunodeficiencies are treated by transplanting bone marrow or fetal thymus tissue. The deficiency of cellular immunity can be weakened by the use of special colony-stimulating factors. For enzymopathies, therapy is carried out using missing enzymes or metabolites - for example, biotin preparations.

For dysglobulinemia (primary humoral immunodeficiency), replacement therapy is used - the introduction of immunoglobulins of the missing classes. In the treatment of any form, it is extremely important to pay attention to the elimination and prevention of infections. At the first signs of bacterial, viral or fungal infection, patients are prescribed a course of appropriate medications. Often for complete cure infectious pathologies require increased dosages of medications. All vaccinations for children are canceled - in most cases they are ineffective, and some are even dangerous.

Prognosis and prevention

The prognosis of primary immunodeficiency varies greatly among different types of pathology. Severe forms may be incurable and lead to death in the first months or years of a child’s life. Other species can be successfully controlled by replacement therapy or other treatment methods, only slightly worsening the patient’s quality of life. Mild forms do not require regular medical intervention, however, patients should avoid hypothermia and contact with sources of infection, and if there are signs of viral or bacterial infection, consult a specialist. Prevention measures, given the hereditary and often congenital nature of primary immunodeficiencies, are limited. These include medical and genetic counseling of parents before conceiving a child (in case of a family history) and prenatal genetic diagnosis. During pregnancy, women should avoid contact with toxic substances or sources of viral infections.

A dangerous disease that is difficult to treat is secondary immunodeficiency. It is not a consequence genetic predisposition and is characterized by a general weakening of the body and immune system. Immunology defines secondary immunodeficiencies as an acquired pathological disorder in the functioning of our body's defenses.

What does secondary immunodeficiency mean?

If we consider secondary immunodeficiency in more detail, what it is in adults, we can give a definition formulated by the branch of general medicine that 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 cause;
• 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, is combined into 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 difficult to treat chronic inflammation defined as clinical manifestations 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 (, poisoning);
• severe psychological and physical injuries, increased physical activity;
• bleeding, burns;
• chemical influences (drugs, steroids, chemotherapy);
• natural factors (old age or childhood, period of gestation);
• 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 health, 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.

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