Since 1978, the list of diseases of the immune system has included another rare variety of pathologies called general variable immunodeficiency or OVID. The causes of this pathology have not yet been fully studied. Fortunately, OBI is extremely rare. According to statistics, 4 men and 15 women per one million people fall ill with unspecified total variable immunodeficiency. What is it, consider in more detail below.
Signs of OVID
Pathology can occur in representatives of both sexes, regardless of age. Most often, however, the age of patients with OVID is in the range of 10-30 years. General variable immunodeficiency in children in the early years of life is rare.
The most characteristic sign of the presence of such a condition is regular infectious diseases of the ENT organs, including the lungs and bronchi. In some cases, patients develop bronchiectasis, that is, irreversible changes in the lungs, characterized by expansion and scarring of the airways. Such pathologies appear under the influence of harmful bacteria such as staphylococci and pneumococci. The main symptom of damage to the lungs and bronchi is a persistent cough with sputum production. This is a reason to check for the presence of general variable immunodeficiency.
Enlarged lymph nodes
Examination and diagnosis of the disease quite often shows enlarged lymph nodes in the chest and neck. This is due to the presence of an infectious disease, as well as a violation of the protective properties of the body. This also explains the enlarged spleen and nodular formations located in the intestinal walls.
Low level of immunoglobulin in the blood composition, as well as the amount of antibodies, is characteristic of insufficiency of general type of immunity. In addition, certain antibodies have the ability to attack cells and tissues in the body. These cells are called autoantibodies.
Many patients undergoing treatment for an infectious disease of a bacterial nature are diagnosed with general type of immune deficiency based on a blood test. Analysis will show progressive anemia caused by the death of red blood cells and a very low platelet count.
What other symptoms of general variable immunodeficiency may be?
Endocrine system diseases
Under the influence of autoantibodies in many patients, diseases of the endocrine system associated with disorders of the thyroid gland, as well as polyarthritis, which is characterized by an inflammatory process in the joints, are manifested. Large joints, such as the knee, elbow, or ankle, are most commonly affected. Small joints are not affected by autoantibodies. Also, harmful cells do not enter the composition of the joint fluid.
Sometimes patients with mild variable immunodeficiency of moderate severity complain of abnormalities in the gastrointestinal tract, such as pain in the stomach, gas, digestive upset, etc. In this case, the test results will indicate a malfunction in the absorption of carbohydrates and fats .
Often, a biopsy of the mucous membrane is performed, as well as a study of feces, which reveals the presence of parasitic invasion, namely lamblia. It is these parasites that can lead to disorders like VID (general variable immunodeficiency). In this case, getting rid of parasites normalizes the patient's condition. If untreated, the disease will progress.
Types of pathology
General variable immunodeficiency refers to a heterogeneous group of diseases. Its main types: congenital, acquired, sporadic, family (with a variable type of inheritance). Unspecified immunodeficiency also occurs.
Diagnostics
Diagnostic measures for the detection of total variable immunodeficiency suggest the following:
- Collection of medical history and patient complaints. During the survey, the doctor finds out when recurrent colds and infectious diseases began, as well as problems with pathological processes in the ENT organs.
- Assessment of life history. The specialist pays particular attention to the developmental suitability of the child's age. Finds out what the patient was sick with in childhood and whether colds and chronic pathologies were common.
- Physical examination. It involves a thorough examination of the nasopharynx in order to detect hyperemia, as well as enlarged tonsils in the sky. Attention is drawn to the patient's eyes, which may turn red and watery. Listening to the lungs can reveal wheezing of various types and hard breathing. Also, a change in the shape of the fingers often appears when there is an extension to the phalanges at the tips, while the nails can take the form of an oval, they become very convex.
- Immune status. It is determined by the analysis of venous blood. The study shows the level of certain classes of immunoglobulins (protective proteins that have a protective effect on the body from attacks of bacteria, fungi and viruses). Also, based on the analysis, the number of B cells is detected, which provide the production of immunoglobulins.
- Check for impaired production of specific antibodies. It is carried out by recording the reaction to the introduction of a special vaccine designed to protect the body from infections. Against the background of immune deficiency, antibodies are not produced by the vaccine.
Diagnosis of general variable immunodeficiency can begin with a visit to a therapist who will give directions to all the necessary tests and refer you to the right specialists.
Treatment
Therapy of pathology proceeds according to the same scheme as other autoimmune pathologies. If OVID is not accompanied by a defeat of T-lymphocytes, then substitution therapy aimed at replenishing immunoglobulins in the blood will help to eliminate the severity of symptoms. For this, special preparations are introduced into the body based on the blood serum of healthy people, which contains IgG immunoglobulins, including all necessary antibodies.
Antibiotic therapy
If the deficiency of the immune system is accompanied by sinusitis or a pulmonary disease in a chronic form, in addition to the immunosubstitution treatment of general variable immunodeficiency, a broad-spectrum antibiotic therapy is given. Usually the course is longer than in healthy people.
Penicillins (Flemoxin, Ampicillin, Flemoclav, Amoxil), cephalosporins (Cefixime, Cefuroxime), as well as macrolides and fluoroquinolones can be used.
Physiotherapy
With bronchiectasis, the physiotherapy procedures have a beneficial effect on the healing process. Also, patients are assigned to perform special exercises aimed at removing sputum from the respiratory tract.
If the problems are due to poor absorption in the intestines of nutrients, the patient is checked for parasites. Next, anthelmintic therapy is performed. Arthritis and polyarthritis are treated with the introduction of immunoglobulin preparations.
Complications
Even the timely detected state of immunodeficiency can lead to a number of complications and consequences, including:
- The formation of bronchiectasis, which is an irreversible expansion of the bronchi, which can subsequently lead to depression of the respiratory function when the body does not receive enough oxygen and, as a result, the functioning of all systems and organs deteriorates.
- Fatal outcome as a result of a severe infection of bacterial origin.
- The formation of malignant tumors can also be the result of an immunodeficiency state - both intrauterine and congenital. This is due to defects at the genetic level, when the immune system does not cope with its functions.
- The development of an autoimmune process, when the body's defense system begins to perceive its cells as foreign, gradually destroying them.
Genetic predisposition
There are a number of assumptions about why a variable type of general immunodeficiency occurs. According to one of them, there is a genetically determined predisposition to the development of this condition. The type of inheritance is not yet clear, but scientists have discovered the absence of certain types of immunoglobulins in members of the same family. To date, studies of mutations at the gene level are being carried out, however, so far they have not been able to confirm or deny the genetic theory of the appearance of OVID.
Forecast
Timely treatment, consisting in the use of drugs based on immunoglobulins and antibacterial treatment, provides a positive prognosis for patients with acute respiratory infections. The main goal of treatment is to ensure the maximum degree of patient protection against infectious lesions and the prevention of chronic manifestations of diseases.
An important feature of the success of treatment of general variable immunodeficiency is the condition in which the patient is referred to a specialist, that is, the neglect of the disease. In general, maintenance therapy for patients with acute respiratory viral infections continues throughout life. A person will need constant administration of immunoglobulins, and even with minor manifestations of an infectious disease, it is necessary to conduct a course of antibacterial therapy.
Patients with OBI are advised to consult a doctor in case of diarrhea or recurrence of infection. It is important to observe a gentle regime and try to take preventive measures to prevent a weakening of the immune system. Most often, patients with a similar diagnosis confirm their partial or complete disability.