Inborn errors of immunity: features of epidemiology and continuity of medical care in the Republic of Tatarstan

Rationale: Inborn errors of the immune system represent the field of modern medicine, in particular clinical immunology, which demonstrates a high rate of accumulation of new data on this problem, which is realized in the current change in ideas about the prevalence of these diseases, revision of classification, and introduction of new treatment methods. Accordingly, many classical paradigms regarding primary immunodeficiencies (PID) have now been revised: taking into account more effective and early diagnosis, as well as modern treatment options, the prognosis of patient survival has largely changed in a positive direction, and the traditional idea of ​​the predominance of patients with early PID childhood is no longer relevant. Objective: to assess the effectiveness and level of continuity of medical care in the Republic of Tatarstan based on an epidemiological analysis of a group of patients diagnosed with PID. Methods: analysis of the register of patients living in the Republic of Tatarstan with an established diagnosis of PID, observed from 1994 to 2023. A total of 240 patients were included in the study. Results: An epidemiological analysis of primary immunity errors in the Republic of Tatarstan (RT) revealed a prevalence of 6.0 per 100,000 population, with a high proportion of patients over 18 years of age (53%), with the largest group of patients having defects in antibody production (42 %). Conclusions: the increasing relevance of the problem of PID requires special attention to the organization of medical care for patients with PID, increasing continuity between specialized pediatric services and the adult network, as well as developing issues of interdisciplinary interaction