Ураження суглобів у пацієнтів з первинними імунодефіцитами

Abstract

Purpose - to analyse the leading markers of primary immunodeficiencies in children with joint syndrome based on a clinical case study. The article presents literature data on one of the immunopathological syndromes - autoimmune syndrome - in children with primary immunodeficiencies. Mechanisms of autoimmunity and frequency of joint lesions are discussed. It is emphasized that mostly lesions of the musculoskeletal system are observed in patients with primary disorders of antibody formation and in certain primary combined immunodeficiencies. It was noted that there are specific features of damage to the musculoskeletal system in various nosological units of primary immunodeficiencies. Basic ideas about modern opportunities and problems of treatment of joint lesions in children with primary immunodeficiencies are given. The main clinical manifestations of Nijmegen syndrome in children are described. A case of joint damage in a child with Nijmegen syndrome is given. Conclusions. The system of treatment of autoimmune pathology in the setting of primary genetically determined disorders of the immune system requires further improvement, given the increasing survival rate of patients with primary immunodeficiencies, and in recent years there is a need to develop consensus recommendations for their management, which will improve their quality of life. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.