• Dimitris Kounatidis, Georgios Kontos, Evangelia Kotsi, Pinelopi Kaparou, Elena Avgoustou, Natalia Vallianou, Melanie Deutsch, and Dimitrios Vassilopoulos.
• 2nd Department of Internal Medicine, Hippokration General Hospital, Athens, Greece. Electronic address: dimitriskounatidis82@outlook.com.
• Am. J. Med. Sci. 2024 Aug 1; 368 (2): 162166162-166.

AbstractAutoimmune polyglandular syndrome (APS) is a rare group of immune-mediated disorders, which are typically, but not exclusively, related to the presence of endocrine abnormalities. APS type 2 is the most common subtype of the syndrome, more often observed in adulthood, with a characteristic clinical triad, which includes adrenal insufficiency, autoimmune thyroiditis and diabetes mellitus type 1. Adrenal insufficiency is an essential and necessary clinical manifestation of the syndrome, as it is observed in 100 % of the cases, while it can be accompanied by hyperchloremic metabolic acidosis. Herein, we present a 23 years-old patient with adrenal insufficiency in the context of autoimmune polyglandular syndrome type 2 with coexisting autoimmune thyroiditis and metabolic acidosis with an increased anion gap attributed to prolonged malnutrition. Additionally, we analyze the main clinical features of adrenal insufficiency, which is a central component of autoimmune polyglandular syndrome; highlight characteristics that differentiate the major APS subtypes.Copyright © 2024 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

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