POS1374 RHEUMATOLOGIC DISEASES AS PRESENTATIONS OF PRIMARY IMMUNODEFICIENCY DISEASES

Background: Primary immunodeficiency diseases (PID) occur on average with a frequency of 1: 100 000 the population. In accordance classification PIDS International Union Immunological Societies (IUIS) 9 different groups innate errors immunity are distinguished [1]. The polymorphism clinical picture, difficulties in recognizing PID cause their late diagnosis and associated development irreversible organ damage, complications high mortality. According to traditional view, marker is an infectious syndrome. However, some cases, detected as rheumatologic disease. Objectives: aim study analyze adult patients living one subjects Russia –Chuvashia. Methods: material was patient data obtained during retrospective analysis 49 outpatient records included Republican Register PID. Diagnosis various forms carried out criteria developed by IUIS Results: During period from 1993 January 2020, cases were registered population Chuvashia. PID, сommon variable (CVID) most common Chuvashia (26 people). second place there selective IgA deficiency (10 people); third X-linked agammaglobulinemia (4 people) hereditary angioedema remaining account for 5 cases: 2 Louis-Bar syndrome, 1 case DiGeorge Wiskott-Aldrich syndrome Hyper IgE main symptom 35 (71.4%) heightened susceptibility infection. 14 (28.6%) patients, picture dominated non-infectious presentations (autoimmune, lymphoproliferative oncological diseases). (18.3%), it manifested (rheumatoid arthritis, psoriatic scleroderma-like syndrome). These symptoms more characteristic CVID. With agammaglobulinemia, rheumatic observed only isolated cases. CVID debuted rheumatoid resulting delay 5.2 years. Only detailed immunological genetic made possible diagnose prescribe adequate treatment – replacement immunoglobulin therapy. This reduced manifestations disease did not significantly improve course rheumatological diseases. Therefore, use methotrexate targeted therapy continued. Conclusion: predominate 28.6% which requires thorough examination order timely manner. References: [1]Picard C, Bobby Gaspar H, Al-Herz W, et al. J Clin Immunol . 2018;38(1):96-128. Disclosure Interests: None declared