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Introduction. Primary immunodeficiencies (PID) are congenital defects of immune system that may manifest as an increased predisposition to infections and autoimmune diseases. Objective: to analyse the spectrum and incidence of autoimmune manifestations in the group of patients with the most common PID — antibodies deficiency. Methods: evaluation of clinical and anamnestic data in 120 patients with primary antibody deficiency. Results. Autoimmune manifestations were found in 33 out of 120 patients (27.5%), with different rate in all groups of primary antibody deficiencies — from 6.25% in patients with IgG subclasses deficiency to 75% with hyper-IgM syndrome. The spectrum of autoimmune pathology included chronic oligoarthritis, celiac disease, uveitis, rheumatic fever, autoimmune neutropaenia, autoimmune haemolytic anaemia, autoimmune thyroiditis, erythema nodosum, fibrous alveolitis, alopecia, and glomerulonephritis. Chronic juvenile arthritis as a manifestation of autoimmune pathology was observed only in patients with severe hypogammaglobulinaemia. Substitution therapy with immunoglobulin preparations in these patients had a significant positive effect on the course of autoimmune diseases. In patients with selective IgA deficiency, celiac disease was most common (in 15% of patients), which was at the forefront among other clinical manifestations. Conclusions. Autoimmune pathology often complicates the course of primary antibody deficiencies, including a wide range of autoimmune diseases, includes a wide range of diseases, and may also be the first manifestation of immunodeficiency. Early diagnosis of primary immunodeficiency and autoimmune diseases in such patients is important for the initiation of adequate therapy and prevention of irreversible effects.

Primary antibody deficiencies — the largest group of congenital immunodeficiencies. This clinically and genetically heterogeneous group of congenital immune defects includes mild, often asymptomatic IgA deficiency and IgG subclasses and severe agammaglobulinemia with inhibition of production of all immunoglobulines. The most common clinical manifestation — severe or recurrent synopulmonary infections. It is possible the development of autoimmune diseases, enteropathy. Replacement therapy with immunoglobulin — the main method of treatment of hypogammaglobulinemia. Key worlds: primary antibody deficiency, diagnostic, treatment.