Late diagnosis of primary chronic adrenal insufficiency in the framework of autoimmune polyglandular syndrome type 2. Clinical observation

Abstract

Chronic adrenal insufficiency is caused by insufficient secretion of hormones by the adrenal cortex due to dysfunction of one or more links of the hypothalamic-pituitary-adrenal system. If the pathological process is localized in the adrenal glands themselves, causing almost total destruction of the cortical layer (at least 90% of the cortex of both adrenal glands), a clinical picture of primary chronic adrenal insufficiency (1-HH) develops. The most common cause of 1-HN is currently autoimmune destruction of the adrenal cortex, which can occur either in the form of an isolated 1-HH of autoimmune genesis, or be a component of autoimmune polyglandular syndromes (APS), which are a primary autoimmune lesion of several peripheral endocrine glands, and often associated with non-endocrine autoimmune diseases. The most common variant of APS is APS type 2, which is a combination of 1-HH with autoimmune thyroiditis (autoimmune thyroiditis, Graves' disease), or with type 1 diabetes mellitus. Despite the fact that adrenal insufficiency is a rare disease, from 40-60 to 100-110 new cases are detected per 1 million adults per year, its timely diagnosis is very important, because without timely replacement therapy with glucocorticoids and mineralocorticoids, a life-threatening condition develops. condition – acute adrenal insufficiency (adrenal crisis). The most common cause of acute hypocorticism is decompensation or acute manifestation of chronic forms of adrenal insufficiency. The article presents a clinical case of late diagnosis of primary adrenal insufficiency in APS type 2, which led to the development of an adrenal crisis in a 42-year-old patient. Late diagnosis was caused by errors in the interpretation of clinical symptoms, as well as unaccounted for data from the anamnesis, namely, the presence of diabetes mellitus of autoimmune genesis in the patient.

Keywords

• аdrenal insufficiency
• аdrenal crisis
• autoimmune polyendocrine syndrome
• аdrenal glands
• endocrinology
• diagnostics