The immune system plays the role of protector in our body from the penetration of foreign viruses, bacteria, fungi and other parasites. A condition in which the immune system fails for various reasons, and these disorders of immune defense have a long-term pathological nature, is called immunodeficiency (immunodeficiency state, IDS). In addition, with immunodeficiency, the process of renewal of the body is slowed down or completely stopped.
Immunodeficiencies are divided into:
- primary (hereditary, at the genetic level)
- secondary (acquired during life).
Secondary IDS are much more common than primary ones. Chronic infectious diseases are the main symptom of both types of immunodeficiency.
Symptoms of immunodeficiency
Symptoms of primary and secondary immunodeficiency have similar manifestations and signs:
- frequent exacerbations of chronic diseases (upper respiratory tract, arthrosis, arthritis, diseases of the gastrointestinal tract, etc.)
- increased susceptibility to various infections
- chronic weakness, depressed mood, fatigue, sweating
- systematically elevated body temperature up to 37.7
- combination of pathologies of different origins in one disease (Bacterial, viral and fungal infections)
- tenderness and enlargement of lymph nodes
- frequent allergic reactions
Immunodeficiency conditions
The immune system, designed to ensure the biological individuality of the body and, as a result, performs a protective function when in contact with infectious, genetically foreign agents, can fail for various reasons, which leads to a disruption of the body’s defense against microbes and manifests itself in increased infectious morbidity.
There are 4 types of immune system pathology:
- Immunological deficiency due to developmental defects or the action of damaging factors (immunodeficiencies);
- Hypersensitivity, or perverted reactivity, the main form of which is allergy;
- Immunopathology caused by autoaggression;
- Tumors of the immune system.
There are primary and secondary immunodeficiency states.
Primary immunodeficiencies
(PID) are human diseases that are based on a hereditary defect in the immune system. These are rare disorders. There is a failure of individual components of immunity: cellular (DiGiorgio syndrome, Wiskott-Aldrich syndrome), humoral, complementary and phagocytic (chronic granulomatosis).
Such diseases accompany a person throughout his life. As a rule, people suffering from primary immunodeficiencies die from various infections in the first three years. However, there are cases that are not life-threatening and are completely compensated by other components of the immune system, for example, selective deficiency of immunoglobulin class A.
Secondary immunodeficiencies
(SID) are disorders of the immune system that develop in late postnatal life or in adulthood. They are not believed to be the result of any genetic defect. Clinically, they manifest themselves as often recurrent bacterial, fungal, and viral infections that are difficult to respond to traditional methods of treatment.
This type of immunodeficiency states occurs as a result of various environmental influences on the human body: radiation, immunosuppressive chemotherapy, as well as infections such as HIV. Compared to primary ones, secondary ones are much more common and much easier to diagnose.
According to the classification, there are three forms of VID:
Acquired
The most striking example of this form of secondary immunodeficiency is acquired immunodeficiency syndrome (AIDS), which develops as a result of damage to the immune system by the human immunodeficiency virus (HIV).
Induced
For this form, the specific cause that caused its appearance has been identified: X-ray radiation, cytostatic therapy, the use of corticosteroids, trauma and surgery, as well as immunity disorders that develop secondary to the underlying disease (diabetes, liver disease, kidney disease, malignant neoplasms).
Spontaneous
Characterized by the absence of an obvious cause that caused the disturbance in immune reactivity. Clinically, it manifests itself in the form of chronic, often recurrent infectious and inflammatory processes of the bronchopulmonary apparatus, paranasal sinuses, urogenital and gastrointestinal tract, eyes, skin, soft tissues caused by opportunistic (opportunistic) microorganisms. Therefore, chronic, often recurrent, sluggish, difficult to treat with traditional means, inflammatory processes of any localization in adults are considered as clinical manifestations of a secondary immunodeficiency state.
Diagnosis of immunodeficiency
The manifestation of signs of primary immunodeficiency occurs immediately after the birth of a child or after a short period of time.
Secondary immunodeficiency can develop at any stage of life.
Diagnosis of immune deficiency includes:
- initial examination of the patient and collection of a complete medical history
- general blood analysis
- blood chemistry
- genetic and immunological tests (for primary immunodeficiency)
- PCR diagnostics
- virological study
Specialists you should contact if you suspect immunodeficiency:
- immunologist
- virologist
- allergist
Signs of immunodeficiencies
In immunodeficiencies, the main manifestations are severe bacterial, fungal or viral infections. We are not talking about banal colds, but about serious conditions: persistent sinusitis, recurrent pneumonia, multiple abscesses, fungal infections of the skin, mucous membranes and intestines, herpetic rashes. The more severe the stage of immunodeficiency, the more serious the manifestations.
Doctors distinguish five groups of PID depending on the damage to one or another part of the immune system:
- damage to humoral immunity;
- combined;
- phagocytosis defects;
- cellular problems;
- pathology of complement systems.
PID can be suspected based on a set of certain signs, but the diagnosis is confirmed in the laboratory, according to an immunogram, and in the case of HIV, by identifying antibodies to human immunodeficiency. Key features include:
- presence of PID in family members, death from infections at an early age;
- recurrent purulent otitis (more than 8 cases per year), two or more sinusitis;
- two or more pneumonias in a year;
- lack of effect from taking standard antibiotics used for more than 2 months;
- severe complications after vaccination with live vaccines;
- recurrent purulent processes of the skin and soft tissues (boils, abscesses);
- identification of two or more systemic infections with meningitis, sepsis, osteomyelitis;
- recurrent systemic candidiasis in patients older than one year;
- severe atypical infections (for example, Pneumocystis pneumonia).
These phenomena can complement long periods of low-grade fever, repeated infections with the transition to chronic, recurrent forms, ineffectiveness in the treatment of infections according to standard regimens, decreased performance, and constant asthenia. But a full diagnosis with an examination by an immunologist and the study of a detailed immunogram is important. Source: RajeN, DinakarC. OverviewofImmunodeficiencyDisorders // ImmunolAllergyClinNorthAm. 2015 Nov;35(4):599-623. doi: 10.1016/j.iac.2015.07.001. Epub 2015 Aug 25..
Objectives of immunological examinations:
- confirmation of immunodeficiency states;
- determination of the severity of violations of one or another part of the immune system;
- individual selection of immunocorrective therapy and assessment of its effectiveness;
- making a prognosis, monitoring therapy, preventing complications.
Treatment
The manifestation of signs of immunodeficiency in children and adolescents indicates a congenital form of the disease, and in an adult – an acquired form.
Treatment of primary immunodeficiency is much more complex and represents a comprehensive approach:
- Establishing an accurate diagnosis with identification of the part of the immune system that has been disrupted.
- Replacement correction of a missing link in the immune system (for example, bone marrow transplantation).
- Administration of immunoglobulins - intravenously or subcutaneously (contraindicated in patients with heart failure).
- Treatment with antibiotics, antifungal or antiviral drugs if infectious complications occur.
Treatment of the second type must begin with identifying and eliminating the cause of its occurrence. Then the immunologist prescribes: antibiotics, immunotherapy and vitamin therapy.
Secondary immunodeficiency - symptoms and treatment
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 - immune system proteins 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, such as zinc, iodine, lithium, copper, cobalt, chromium, molybdenum, selenium, manganese and iron. Their lack aggravates immune dysfunction [5].
Due to a lack of vitamins, an imbalance of macro- and microelements caused by an unbalanced diet, the functioning of the cellular immune system is disrupted: the response of lymphocytes to mitogens that stimulate T-cells is reduced, lymphoid tissue atrophies, and the function of neutrophils (blood cells that suppress harmful substances) is impaired [ 5].
The mechanism of development of secondary immunodeficiency can be most clearly demonstrated using the example of viruses of the herpetic group. Some viruses (for example, herpes simplex viruses, cytomegalovirus, and Epstein-Barr virus) reside permanently in the body's cells. Periodically activated under the influence of stress, unbalanced nutrition, concomitant pathology or immunosuppressive therapy, they contribute to the development of various clinical manifestations [3].
Tumors that arise in the body with weakened immune defenses deplete the immune system as they grow, and the treatment (surgery, radiation and chemotherapy) aggravates the resulting immunodeficiency. In this case, the cellular component of immunity suffers:
- the number of T-lymphocytes, which destroy cancer and infected cells, decreases;
- the activity of suppressor cells that control the strength and duration of the immune response increases;
- phagocytes begin to more slowly absorb bacteria, viruses, dead or dying cells [6].
Many chronic diseases also lead to the development of secondary immunodeficiency. For example, in diabetes mellitus, chemotaxis (the movement of microorganisms in response to a chemical substance) and phagocytic activity of neutrophils (cells involved in the capture of viruses and bacteria) are inhibited, protection against bacterial infections is impaired, as a result of which skin pyoderma (purulent diseases) and abscesses develop [7 ].
The body responds to stress by releasing active substances such as catecholamines and glucocorticoids, which affect the functioning of immune system cells. Under their influence, pro-inflammatory cytokines are released, processes are launched aimed at combating a possible infectious agent. With chronic stress, due to prolonged exposure to glucocorticoids on cells of the immune system, the level of immunoglobulins, the activity of T-cell immunity and phagocytosis decreases. As a result, the cells of the immune system are damaged: the process of their oxidation and programmed death begins. If there was no excess load and the body was sufficiently rested, then the functioning of the immune system is normalized [9].
Carrying out immunotherapy
After a complete diagnosis has been completed and the type of immunodeficiency has been determined, the immunologist selects an individual treatment regimen.
The use of immunotherapy (drugs for the correction of immunity) is aimed at strengthening the functioning of the immune system, correcting the imbalance of immune reactions, suppressing the aggression of autoimmunity, and pathologically active processes. It is also important to stimulate the parts of immunity that are impaired in a particular patient.
Prof. Ignatiev V. A.
Sources:
- M. Yesenaliev. Acquired immunodeficiency syndrome is the main problem of public health // Bulletin of AGIUV, 2013, No. 1, pp. 64-65.
- Yu.N. Fedorov, V.I. Klyukina, M.N. Romanenko, O.A. Bogomolova, A.N. Denisenko. Strategy and principles of immunocorrection and immunomodeling therapy.
- Raje N, Dinakar C. Overview of Immunodeficiency Disorders // Immunol Allergy Clin North Am. 2015 Nov;35(4):599-623. doi: 10.1016/j.iac.2015.07.001. Epub 2015 Aug 25.
