Autoimmune Polyglandular Syndrome Type II Presenting as an Endocrine Emergency: A Case Report

Abstract

We describe the case of a 30-year-old male, a known patient of type 1 diabetes mellitus (DM) on insulin therapy, seeking medical attention for recent onset repeated attacks of hypoglycemia associated with generalized weakness and darkening of skin. Further evaluation and screening revealed autoimmune adrenal failure together with presence of Hashimoto's thyroiditis. The patient was diagnosed as a case of autoimmune polyglandular syndrome (APS) type II with complete triad of Addison's disease, type 1 DM and autoimmune thyroid disease. Anti-thyroid peroxidase, anti-glutamic acid decarboxylase and anti-endomysial antibodies were present in our patient. He was started on replacement therapy with physiological dose of prednisolone and thyroxine resulting in marked improvement in his symptoms. Recurrent hypoglycemia in a type 1 DM patient should raise a suspicion of underlying autoimmune adrenal insufficiency. Absence of obvious signs of thyroid dysfunction also poses a diagnostic challenge for the clinicians. This article aims at highlighting the importance of detailed evaluation together with long term followup of these patients and their relatives as overt clinical disease may only be the tip of the iceberg of other underlying organ-specific autoimmune diseases that may develop later in the course.

Keywords: Addison’s disease; Autoimmune polyglandular syndrome; Autoimmune thyroid disease; Hypoglycemia; Type 1 diabetes mellitus.

Figure 1

The knuckles of hand, oral mucosa and tongue shows dark hyperpigmented patches suggestive of adrenal insufficiency.

Figure 2

Contrast enhanced CT scan of abdomen shows bilateral morphologically normal adrenal glands.