Autoimmune polyglandular syndrome type 2 in the practice of an endocrinologist

Abstract

Autoimmune polyglandular syndromes are a group of rare multi-organ pathologies resulting from autoimmune aggression and characterized by polymorphic endocrine and non-endocrine organ lesions. Depending on the combination of organs involved in the autoimmune process, there are 4 types of autoimmune polyglandular syndrome. Type 1 has an autosomal recessive type of inheritance, more common in children and adolescents. Types 2–4 are associated with the expression of antigens the HLA system and manifest typically in adult patients. The article provides a brief description of all types of autoimmune polyglandular syndromes, in more detail describes type 2 (Schmidt syndrome), the clinical case of which is addressed in this article. The following is a clinical case: observation of a 46-year-old male hospitalized in the endocrinological department of Samara City Hospital No. 5 with autoimmune polyglandular syndrome type 2 with decompensation of adrenal insufficiency and hypothyroidism. Submitted complaints, anamnesis, laboratory and instrumental examination of the patient, results of screening for the presence of antibodies that confirm the diagnosis of autoimmune polyglandular syndrome type 2. Surveys have been conducted to eliminate other causes of primary adrenal insufficiency. Additional surveys carried out to identify other possible components of autoimmune polyglandular syndrome type 2 are described. The prescribed treatment according to the nationalclinical recommendations, as well as the features of the prescription of hormone replacement therapy, described further dynamic observation at the outpatient stage and given laboratory control indicators. Conclusions are made about possible difficultiesin the diagnosis and treatment of this pathology.