Treatment of immunodeficiency Gorky. Human immunodeficiency (primary, secondary), causes and treatment. Objectives of immunological research

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Immunodeficiency is a dysfunction of the immune system, which manifests itself in a decrease in the body's resistance to various viruses, bacteria, and fungi.

There are 2 forms of immunodeficiency:

• congenital;
• acquired.

Factors that predispose to the development of this condition:

• severe infectious or viral diseases (HIV, tuberculosis, viral hepatitis);
• oncological diseases;
• autoimmune diseases (aplastic anemia);
• conditions that lead to depletion of the body (vitaminosis, stress, depression, microwave radiation);
• diabetes mellitus, hormonal imbalance;
• injuries, surgical interventions.

The symptoms are very diverse, as the disease is disguised as other pathologies.

When the respiratory tract is affected, the following are observed:

• cough, runny nose, fever;
• weakness, headaches.
• Lesions of the digestive tract are characterized by:
• vomiting, nausea;
• headache, dizziness;
• stomach bleeding;
• temperature increase;
• pain in the intestines.

CNS damage is indicated by:

• headache;
• temperature increase;
• convulsions.

Common typical symptoms of immunodeficiency are:

• pneumonia, which is difficult to treat;
• temperature increase;
• diarrhea for more than 3 months;
• candidiasis.

Diagnostic tests

It is quite difficult to identify this pathology. This is possible only after a comprehensive examination, which includes:

• clinical analysis of blood, urine;
• immunological analysis of immunoglobulins E, A, G, M;
• detection of viral hepatitis C, B;
• blood test for HIV;
• chest x-ray;
• CT scan of affected organs.

Main stages of treatment

Before starting replacement therapy, which is carried out for life (with the help of donor plasma, serums, etc.), it is necessary to get rid of concomitant infectious diseases. For this purpose, broad-spectrum antibiotics, antiviral therapy, and antifungal drugs are used. Immunostimulating therapy (Cycloferon, Inflamafertin) is also carried out. It is recommended to take vitamin-mineral complexes and nutritional supplements. Antidepressants are prescribed according to indications. It is necessary to observe the correct work and rest schedule, give up bad habits - smoking, drinking alcohol.

The most effective treatment is a bone marrow transplant. But it is carried out only after other methods have not helped.

Complications

If left untreated, immunodeficiency contributes to the development of infectious processes (sepsis, pneumonia), which are very difficult to treat and can be fatal.

The human immune system is designed to respond in a timely manner to the invasion of foreign elements. Its correct functionality is to recognize the threat and destroy it. Primary immunodeficiency means that the child did not develop a protective mechanism during intrauterine development, or he did not receive it due to a hereditary factor. As a result, harmful microorganisms entering his body will cause him maximum harm. The same can be said about atypical cells, which have a negative impact on health and cause pathologies of varying severity.

It is necessary to distinguish between primary and secondary immunodeficiencies. Primary is determined in the baby shortly after birth. His body is deprived of the ability to protect itself from antigens and is susceptible to infectious invasion. This is expressed in the fact that the baby often gets sick, he is overcome by repeated illnesses, he finds it difficult to bear them, and gets complications. Severe forms of primary immunodeficiency lead to death in infancy.

There are rare cases where primary immune deficiency manifested itself in adults. This is possible, but for this a person must have high compensation for a certain type of disease.

The clinical picture of the disease is re-infection, the transition of diseases into a chronic form. What does primary immunodeficiency lead to:

1. The patient suffers from bronchopulmonary anomalies.
2. It affects the mucous membranes and skin.
3. There are problems with the ENT organs.
4. PIDS usually leads to lymphadenitis, abscesses, osteomyelitis, meningitis, and sepsis.
5. Certain forms of primary immunodeficiency provoke allergies, autoimmune diseases, and the growth of malignant neoplasms.

Immunology is the study of dysfunctions of the immune defense - the science of the development and formation of a protective mechanism that counteracts the penetration of antigens into the body and destroys cells damaged by harmful substances and microorganisms.

The earlier PIDs is diagnosed, the greater the child’s chances of surviving and continuing life in satisfactory health. It is important to timely determine the gene mutation, which makes it possible to decide on family planning.

Immunodeficiency is a persistent abnormality of the defense mechanism, which causes a malfunction in the immune response to the influence of antigens. This failure can be of four types:

• age-related, that is, arising in childhood or old age;
• acquired due to poor diet, lifestyle, medication, AIDS virus, etc.;
• developed as a result of various infections;
• congenital or primary ID.

PIDs are classified depending on the forms and severity of the disease. Primary immunodeficiencies include:

• ID characterized by damage to several cellular complexes;
• Reticular dysgenesis, in which stem cells are absent, dooms the newborn to death.
• Severe combined ID is a hereditary disease caused by dysfunction of B and T lymphocytes.
• DiGeorge syndrome - or abnormalities of the thymus, parathyroid glands - underdevelopment or absence of the thymus gland. As a result of the defect, T-lymphocytes are affected, congenital heart defects, deformations in the bone structure, the structure of the facial bones, kidney defects and central nervous system dysfunction occur.
• Primary immunodeficiency caused by damage to B lymphocytes.
• Disorders in myeloid cells causing chronic granulomatous disease (CGD) with an abnormality in oxygen metabolism. Defective production of active oxygen leads to chronic fungal and bacterial infections.
• Defects in complex blood proteins that impair humoral defenses. The complement system may be missing several components.

Need to know! Cellular immunodeficiency is characterized by a deficiency of immunocompetent cells, which include lymphocytes, plasma cells, and macrophages. Humoral immunodeficiency means dysfunction in the production of antibodies.

Symptoms of primary immunodeficiencies

Primary immunodeficiencies are indicated by signs and symptoms. By studying the clinical picture of the disease, the clinic’s doctors identify a type of immune deficiency. This is facilitated by examination, tests, and history taking to determine genetic pathology.

1. Primary deficiencies of cellular immunity give rise to viral and fungal infections. Characteristic signs are repeated colds, severe acute respiratory viral infections, chickenpox, mumps, and frequent manifestations of herpes. The patient suffers from thrush, inflammation of the lungs and gastrointestinal tract caused by fungi. Cellular immunodeficiency increases the risk of cancer and lymphoma.
2. Insufficient humoral protection is provoked by bacterial infections. These are pneumonia, ulcers on the skin, erysipelas, staphylococcus, streptococcus.
3. Insufficiency of the level of secretory immunoglobulin A causes damage to the mucous membranes in the mouth, nose, eyes, intestines, and the bronchi suffer.
4. Combined IDs are characterized by complications of viral and bacterial infections. Manifestations of this form of primary immunodeficiency are nonspecific - they are expressed in developmental defects, tumor processes, lymphoid tissues, thymus gland, megaloblastic anemia.
5. Congenital neutropenia and dysfunction of phagocytosis of granulocytes gives rise to bacterial inflammatory processes with ulcers and abscesses. The result may be sepsis.
6. Complement-associated primary immunodeficiencies lead to bacterial infections, autoimmune diseases, as well as recurrent swelling of the body and limbs - hereditary angioedema (HAE).

Causes of primary immunodeficiencies

Dysfunctions of the immune system develop in the embryo inside the mother's womb. This process is influenced by various factors. Prenatal diagnosis shows a combination of congenital malformations of the fetus with immunodeficiency. The etiology of PIDs is based on three pathologies.

1. Genetic mutations, meaning that changes have occurred in the genes on which immunocompetent cells perform their functions. That is, the process of cell development and differentiation is disrupted. The anomaly is inherited in an autosomal recessive manner, when both parents are carriers of the mutagen. Only a small number of mutations develop spontaneously or germinatively (in germ cells).
2. A teratogenic factor is the influence of dangerous toxins on the embryo, leading to congenital primary immunodeficiency. ID is provoked by TORCH infections - cytomegalovirus, herpes, rubella, toxoplasmosis in pregnant women.
3. Unclear etiology. Immune deficiency, the cause of which is not clear.

Such conditions include asymptomatic ID, which manifest themselves as infectious complications in provocative situations. If even one of the elements of the defense mechanism undergoes an anomaly, then the protective forces are weakened, the patient becomes an object for the invasion of various infections.

Diagnosis of primary immune deficiency

Immunodeficiency conditions are identified by type, since primary ID is most often congenital, its type is determined in the first months or weeks. A visit to the doctor is required if the baby is frequently ill, colds, or the development of fungal, viral, or bacterial infections. Anomalies in the development of a child may also depend on primary immunodeficiency. To solve the problem, urgent diagnosis and immediate initiation of treatment are necessary.

The disease identification method includes the following procedures:

• general examination, during which attention is paid to damage to the skin, mucous membranes, pustular processes, subcutaneous swelling of adipose tissue;
• study of the leukocyte formula using a general blood test, ID is indicated by the presence of leukopenia, neutropenia, agranulocytosis, and other disorders;
• blood biochemistry shows dysgammaglobulinemia, the presence of uncharacteristic metabolites, indicating primary humoral ID;
• a specific study on the reactions of the immune system. Indicators of activity of immunocompetent cells are studied;
• molecular genetic analysis - a method of gene sequencing for the type of mutation. This is a way to determine Bruton, DiGeorge, Duncan, and Wiskott-Aldrich syndromes.

The doctor differentiates immunodeficiency states from acquired secondary IDs arising from the influence of radiation, toxic substances, autoimmune diseases, and oncology. In adults, the diagnosis is difficult to make, since the signs are smoothed out and the symptoms are unclear.

Prenatal diagnosis

Determination of primary ID using chorionic villus biopsy is called prenatal identification of the form of the disease. In addition, the culture of cells of fetal fluid and fetal blood is being studied. These are complex tests that are indicated in cases where a mutagen has been detected in parents.

But for the detection of X-linked severe combined immunodeficiency, this method gives an accurate result, and also clarifies the diagnosis for primary ID syndromes, chronic granulomatosis, and other SCID conditions.

Treatment of primary immunodeficiencies

The different etiologies and pathogenesis of diseases do not allow us to develop a general method of treating pathology. In severe forms, therapeutic treatment is not relevant, it brings only temporary relief, but death is inevitable from complications of immunodeficiency. In these cases, only bone marrow or embryonic substance of the thymus transplantation helps.

The deficiency of cellular immunity is compensated by the use of specific colony-stimulating drugs. This is replacement immunotherapy with thymalin, tactivin, levamisole and other agents, the choice of which is made by the immunologist. Enzymepathies are corrected by enzymes and metabolites. A common drug in this series is biotin.

Dysglobulinemia (lack of humoral protection) is treated with immunoglobulin replacement, depending on the missing substances of this type. But the main obstacle to the progression of the disease is the prevention of infections. Moreover, vaccination of children with primary ID does not have an effect; it is dangerous.

Prognosis and prevention

With severe primary ID, the child is doomed; he dies in the first year of life. Other pathologies of the immune system are treated as described above. The main task of parents is timely access to a doctor and care for their children. The child should not be allowed to become infected with viral, bacterial, or fungal pathogens.

If you are planning to have a child and have problems with gene mutation, then consultation with an immunologist is mandatory. During pregnancy, you need to undergo prenatal diagnosis, protect yourself from infections and follow all doctor’s recommendations.

For patients with ID, it is important to maintain personal hygiene, care for the oral cavity, nasal mucosa, and eyes carefully, without damaging their integrity. A balanced diet, avoidance of contact with patients during epidemics, and drug prevention of infections are necessary.

Complications after immunodeficiency

Primary immunodeficiencies lead to serious complications. The consequences may result in the death of a person. Such conditions are considered sepsis, abscesses, pneumonia, and severe infections. Autoimmune diseases are possible when the immune system fails and destroys its own cells. The risk of cancer and imbalance of the gastrointestinal tract and cardiovascular system increases.

Conclusion

Primary immunodeficiency is not always a death sentence. You need to be constantly monitored by an immunologist; this will help you maintain a satisfactory quality of life and live a long time.

Immunodeficiency– this is a decrease in the functional activity of the main components of the immune system, leading to a disruption of the body’s defense against microbes, and manifested in increased infectious morbidity.

In the modern world, in a megacity, an immunodeficiency state can develop in any person. The danger of this condition lies in its untimely recognition and treatment, which leads to severe infections, autoimmune diseases and oncological processes.

Immunodeficiency conditions are divided into congenital and acquired or secondary (SID). Basically, we encounter secondary immunodeficiencies, and each of us has experienced this condition at least once in our lives. SID refers to disorders of the immune system that develop in old age and, as is commonly believed, are not the result of any genetic defect.

Forms VIEW

Signs VIEW

Signs of VID by which a doctor or the patient himself may suspect an immunodeficiency state

1. Recurrent virus-bacterial infections, characterized by:

• chronic course;
• incomplete recovery;
• unstable remission;
• unusual pathogens (opportunistic flora, opportunistic infection with reduced virulence, with multiple resistance to antibiotics).

2. Age, presence of blood relatives with primary immunodeficiency;

3. Unusual reactions to live, attenuated vaccines;

4. Upon examination, the patient may be diagnosed with developmental insufficiency or developmental delay, chronic diarrhea, low-grade fever, enlarged or complete absence of lymph nodes of the tonsils, thymus, skin abscesses, dermatitis, mucosal candidiasis, congenital malformation, impaired development of the facial skull, short stature (dwarfism) ), increased fatigue;

5. Iatrogenic interventions: chemotherapy, splenectomy, radiation;

6. Prolonged physical and/or psycho-emotional stress;

7. Allergy;

8. Autoimmune diseases;

9. Tumors.

Objectives of immunological research

• confirm the presence of immunodeficiency;
• determine the severity of violations;
• identify the broken link;
• evaluate the possibilities of selecting an immunocorrector;
• evaluate the prognosis of the effectiveness of immunotherapy.

Immunotherapy

After a full immunostudy, the immunologist prescribes therapy.

Immunotherapy (correction of immunity)- treatment aimed at strengthening weakened immune defenses, correcting imbalances in ongoing immune reactions, weakening pathologically active immune processes and suppressing auto-aggressive immune reactions. Not all types of immune defense are effective against a specific infectious agent, but only some.

It is necessary to stimulate those parts of the immune system that are effective in protecting against a specific infection that the patient has.

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Immunodeficiency - what is it?

Doctors note that patients are increasingly being diagnosed with serious diseases that are difficult to treat. Immune deficiency, or scientifically known as immunodeficiency, is a pathological condition in which the immune system does not work properly. Both adults and children experience the described disorders. What is this condition? How dangerous is it?

Immunodeficiency is characterized by a decrease in activity or the inability of the body to create a protective reaction due to the loss of the cellular or humoral immune component.

This condition can be congenital or acquired. In many cases, IDS (especially if not treated) is irreversible, however, the disease can also have a transitive (temporary) form.

Causes of immunodeficiency in humans

The factors causing IDS have not yet been fully studied. However, scientists are constantly studying this issue to prevent the onset and progression of immunodeficiency.

Immunodeficiency, causes:

The cause can only be identified through a comprehensive hematological diagnosis. First of all, the patient is sent to donate blood to assess cellular immunity indicators. During the analysis, the relative and absolute number of protective cells is calculated.

Immunodeficiency can be primary, secondary or combined. Each disease associated with IDS has a specific and individual severity.

If pathological signs occur, it is important to promptly consult your doctor to receive recommendations for further treatment.

Primary immunodeficiency (PID), features

It is a complex genetic disease that manifests itself in the first few months after birth (40% of cases), in early infancy (up to two years - 30%), in childhood and adolescence (20%), less often - after 20 years (10%).

It should be understood that patients do not suffer from IDS, but from those infectious and concomitant pathologies that the immune system is unable to suppress. In this regard, patients may experience the following:

• Polytopic process. This is multiple damage to tissues and organs. Thus, the patient may simultaneously experience pathological changes, for example, in the skin and urinary system.
• Difficulty in treating a particular disease. The pathology often becomes chronic with frequent relapses (repetitions). Diseases are rapid and progressive.
• High susceptibility to all infections, leading to polyetiology. In other words, one disease can be caused by several pathogens at once.
• The usual therapeutic course does not give the full effect, so the dosage of the drug is selected individually, often in loading doses. However, it is very difficult to cleanse the body of the pathogen, so carriage and a latent course of the disease are often observed.

Primary immunodeficiency is a congenital condition, the beginnings of which were formed in utero. Unfortunately, screening during pregnancy does not detect severe anomalies at the initial stage.

This condition develops under the influence of an external factor. Secondary immunodeficiency is not a genetic disorder; it is first diagnosed with equal frequency in both childhood and adulthood.

Factors causing acquired immunodeficiency:

• deterioration of the ecological environment;
• microwave and ionizing radiation;
• acute or chronic poisoning with chemicals, heavy metals, pesticides, low-quality or expired food;
• long-term treatment with drugs that affect the functioning of the immune system;
• frequent and excessive mental stress, psycho-emotional stress, anxiety.

The above factors negatively affect immune resistance, therefore, such patients, in comparison with healthy ones, will more often suffer from infectious and oncological pathologies.

Main reasons, which may cause secondary immunodeficiency are listed below.

Errors in nutrition - The human body is very sensitive to a lack of vitamins, minerals, proteins, amino acids, fats, and carbohydrates. These elements are necessary to create a blood cell and maintain its function. In addition, the normal functioning of the immune system requires a lot of energy, which comes with food.

All chronic diseases negatively affect the immune defense, worsening resistance to foreign agents penetrating into the body from the external environment. In the chronic course of infectious pathology, the hematopoietic function is inhibited, therefore the production of young protective cells is significantly reduced.

Adrenal hormones. An excessive increase in hormones inhibits the function of immune resistance. A malfunction occurs when material metabolism is disrupted.

A short-term condition, as a defensive reaction, is observed due to severe surgical procedures or severe injury. For this reason, patients undergoing surgery are susceptible to infectious diseases for several months.

Physiological characteristics of the body:

• prematurity;
• children from 1 year to 5 years;
• pregnancy and lactation period;
• old age

Features in people of these categories are characterized by suppression of immune function. The fact is that the body begins to work intensively in order to bear additional load to perform its function or survive.

Malignant neoplasms. First of all, we are talking about blood cancer - leukemia. With this disease, there is an active production of protective non-functional cells that cannot provide full immunity.

Also a dangerous pathology is damage to the red bone marrow, which is responsible for hematopoiesis and replacement of its structure with a malignant focus or metastases.

Along with this, all other oncological diseases cause a significant blow to the protective function, but disorders appear much later and have less pronounced symptoms.

HIV – human immunodeficiency virus. By suppressing the immune system, it leads to a dangerous disease - AIDS. All the patient's lymph nodes are enlarged, oral ulcers often recur, candidiasis, diarrhea, bronchitis, pneumonia, sinusitis, purulent myositis, and meningitis are diagnosed.

The immunodeficiency virus affects the defense response, so patients die from diseases that a healthy body can hardly resist, and even more so when weakened by HIV infection (tuberculosis, oncology, sepsis, etc.).

Combined immunodeficiency (CID)

It is the most severe and rare disease that is very difficult to cure. CID is a group of hereditary pathologies that lead to complex disorders of immune resistance.

As a rule, changes occur in several types of lymphocytes (for example, T and B), whereas with PID only one type of lymphocyte is affected.

CID manifests itself in early childhood. The child does not gain weight well and is delayed in growth and development. These children are highly susceptible to infections: the first attacks may begin immediately after birth (for example, pneumonia, diarrhea, candidiasis, omphalitis).

As a rule, after recovery, a relapse occurs after a few days or the body is affected by another pathology of a viral, bacterial or fungal nature.

Treatment of primary immunodeficiency

Today, medicine has not yet invented a universal medicine that helps to completely overcome all types of immunodeficiency conditions. However, therapy is proposed aimed at relieving and eliminating negative symptoms, increasing lymphocyte protection and improving quality of life.

This is a complex therapy, selected on an individual basis. The patient's life expectancy, as a rule, depends entirely on the timely and regular use of medications.

Treatment of primary immunodeficiency is achieved by:

• prevention and concomitant therapy of infectious diseases in the early stages;
• improving protection by bone marrow transplantation, immunoglobulin replacement, neutrophil mass transfusion;
• increasing lymphocyte function in the form of cytokine treatment;
  introduction of nucleic acids (gene therapy) in order to prevent or stop the development of a pathological process at the chromosomal level;
• vitamin therapy to support immunity.

If the course of the disease worsens, you should inform your doctor about this.

Treatment of secondary immunodeficiency

As a rule, the aggressiveness of secondary immunodeficiency states is not severe. Treatment is aimed at eliminating the cause of IDS.

Therapeutic focus:

• for infections - elimination of the source of inflammation (with the help of antibacterial and antiviral drugs);
• to increase immune defense - immunostimulants;
• if IDS was caused by a lack of vitamins, then a long course of treatment with vitamins and minerals is prescribed;
• human immunodeficiency virus - treatment consists of highly active antiretroviral therapy;
• for malignant tumors - surgical removal of a focus of atypical structure (if possible), chemotherapy, radiotherapy,
• tomotherapy and other modern methods of treatment.

In addition, if you have diabetes, you should carefully monitor your health: stick to a low-carbohydrate diet, regularly test your sugar levels at home, take insulin tablets or administer subcutaneous injections in a timely manner.

Treatment of CID

Treatment for primary and combined forms of immunodeficiency is very similar. The most effective treatment method is considered to be a bone marrow transplant (if T-lymphocytes are damaged).

• Today, transplantation is successfully carried out in many countries to help overcome an aggressive genetic disease.

Prognosis: what awaits the patient

The patient must be provided with high-quality medical care in the early stages of the disease. If we are talking about a genetic pathology, then it should be identified as early as possible by taking many tests and undergoing a comprehensive examination.

Children who suffer from PID or CID from birth and do not receive appropriate treatment have a low survival rate to two years.

In case of HIV infection, it is important to regularly test for antibodies to the human immunodeficiency virus in order to monitor the course of the disease and prevent sudden progression.

According to the World Health Organization, 70% of Russians have reduced immunity. And, as you know, a person with a weakened immune system is extremely sensitive to various infections and has a more difficult time suffering from various diseases. However, in most cases, it is impossible to figure out what the causes are and to cure immunodeficiency without the help of a specialist.

Great health!

Immunodeficiency is a persistent decrease in the function of the immune system and resistance to bacteria and viruses. There are primary immunodeficiencies (congenital) that develop in infancy. Such pathological conditions are extremely rare. It occurs much more often secondary immunodeficiency, which develops against the background of poor ecology, poor nutrition, long-term use of medications, chronic diseases, abuse of alcohol, cigarettes, drugs, constant lack of sleep, severe stress, also affects the development of secondary immunodeficiency, etc.

The immune system also experiences a serious burden in the event of serious diseases (for example, staphylococcus, pneumococcus, herpes, chronic viral hepatitis, rubella, toxoplasmosis). Immunoreactivity is disrupted, increased sensitivity to microbial antigens develops, and intoxication of the body appears. And with a disease such as diabetes, the processes of cell division and differentiation, including cells of the immune system, are disrupted, and due to increased glucose in the blood, harmful bacteria multiply. As a result, susceptibility to harmful pathogens increases significantly, and the patient suffers from constant recurrent infections. Therefore, when serious diseases occur, it is necessary not only to treat the main problem, but also to strengthen the body, otherwise the immune system may develop a “crack,” which subsequently threatens complications.

However, there are diseases (rheumatoid arthritis, systemic lupus erythematosus, systemic scleroderma) that lead to persistent immunodeficiency, which is very difficult to combat, and treatment sometimes lasts for years.

Diagnosis of immunodeficiencies

In the presence of chronic foci of infection, recurrent diseases and the ineffectiveness of standard methods of therapy, secondary immunodeficiency can be assumed. In such a situation it is necessary diagnosis of immunodeficiencies: You should consult an immunologist who will prescribe tests and tests at two levels. Level 1 tests are approximate and help identify gross defects in the immune system. Level 2 tests are functional and are aimed at identifying a specific “breakdown” in the immune system.

Treatment of immunodeficiency

After diagnosing immunodeficiencies and receiving the results, the doctor will develop tactics treatment of secondary immunodeficiency. If the problem arose due to an unbalanced diet, he will prescribe a specific diet in combination with a complex of vitamins, minerals and dietary supplements containing the necessary components. And if immunodeficiency is a consequence of a chronic disease, then therapy will most likely begin with the treatment of a chronic source of infection.