Autoimmune polyglandular syndrome with shock and high anion gap metabolic acidosis

Autoimmune 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...

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Veröffentlicht in:The American journal of the medical sciences 2024-08, Vol.368 (2), p.162-166
Hauptverfasser: Kounatidis, Dimitris, Kontos, Georgios, Kotsi, Evangelia, Kaparou, Pinelopi, Avgoustou, Elena, Vallianou, Natalia, Deutsch, Melanie, Vassilopoulos, Dimitrios
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container_issue 2
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container_title The American journal of the medical sciences
container_volume 368
creator Kounatidis, Dimitris
Kontos, Georgios
Kotsi, Evangelia
Kaparou, Pinelopi
Avgoustou, Elena
Vallianou, Natalia
Deutsch, Melanie
Vassilopoulos, Dimitrios
description Autoimmune 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.
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subjects 21-hydroxylase
Acidosis
Addison disease
APS
Autoimmune polyglandular syndrome
title Autoimmune polyglandular syndrome with shock and high anion gap metabolic acidosis
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