| Abstract [eng] |
Common variable immunodeficiency is a disease characterized by defective immunoglobulin production. It is a primary humoral immunodeficiency in which serum levels of immunoglobulins G, A and /or M are reduced. It is the most common symptomatic antibody deficiency with a prevalence of 1: 25 000. Although some studies have already identified certain genetic factors that contribute to the disease, its root causes are still unknown. The clinical manifestations of this immunodeficiency vary widely: recurrent infections, autoimmune diseases, granulomas, lymphoproliferative and gastrointestinal diseases, and others discussed in this review. Common variable immunodeficiency is determined by a laboratory test for immunoglobulin and a demonstration of a decreased response to vaccination. Other possible causes of hypogammaglobulinemia should be ruled out. The essence of the treatment of this disease is immunoglobulin replacement therapy, which provides passive immunity for these patients. The goal of therapy is to prevent long-term organ damage and increase the quality of life. It has been shown to help prevent recurrent upper and lower respiratory tract infections, which contribute significantly to the morbidity and mortality of these patients. Adequate protection against infections is ensured when serum immunoglobulin G levels are higher than 5 g / l. This review will describe various clinical manifestations and a variety of symptoms of common variable immunodeficiency which will help understand its heterogeneity. Also, recommendations are provided to more efficiently suspect and diagnose the disease. |
