Polyglandular autoimmune syndrome in adults: molecular genetic and clinical characteristics (lecture)

Consilium Medicum

Anna A. Larina¹  Ekaterina A. Troshina¹  Maria A. Terekhova² 

[1] Endocrinology Research Centre, Moscow, Russia;I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia;

关键词: polyglandular autoimmune syndrome;    chronic adrenal insufficiency;    autoimmune thyroiditis;    diabetes mellitus type 1;    hypoparathyroidism;    hypothyroidism;    human leukocyte antigens;   

DOI  :  10.26442/20751753.2019.4.190361

来源: DOAJ

【 摘 要 】

Endocrine system disorders (diabetes mellitus, thyroid gland pathologies, polyglandular autoimmune syndromes, adrenal insufficiency, autoimmune ophthalmopathy and others) in development of which immune response disorders play an important role are among the most severe chronic human diseases prevention and early diagnostics of which are often hampered by disease clinical course latent phases. Polyglandular autoimmune syndromes (PAS) comprise damage of two or more endocrine glands characterized with multiple organ failure. In Russian classification the syndrome is divided into 2 main subtypes: PAS type 1 (the prevalence varies from 1:9 thousands to 1 per 200 thousands population) and PAS type 2 (the prevalence is 1.4–4.5 cases per 100 thousands population). Other classifications comprise hidden (latent and potential) forms of the disorder. Patients with PAS are a special cohort that requires careful monitoring and regular screening for prognosis and detection of new components of PAS. High prevalence and incidence rates, disabling complications, and reproductive losses – all of these are common in autoimmune endocrinopathies and are an important challenge at present. The lecture summarizes key clinical and diagnostic characteristics of PAS, proposes practical approach to these syndromes treatment, and specifies key laboratory markers for the disorder dynamics monitoring.

【 授权许可】

Unknown