Polyglandular Syndrome Type 1 Complicated with Dilated Cardiomyopathy: A Case Report

Abstract

Abstract The autoimmune polyglandular syndrome (APS) is a rare recessive inherited syndrome that presents a discrete pattern of endocrine abnormalities. There are 3 types of APS: type 1, type 2, and type 3. The rarest type is type 1 acute polyglandular syndrome. Type 1 polyglandular syndrome targets endocrine and nonendocrine tissues in an autoimmune-destructive process. Our case report is about a 27-year-old male who came with complaints of fever, dysarthria, and dysphagia and presented with oral candidiasis and nail dystrophy, along with complaints of alopecia since he was 7 years old, progressively worsening with time. In the following year, he was diagnosed with hypoparathyroidism and dilated cardiomyopathy. Laboratory results display hypocalcemia, hyperphosphatemia, low Parathyroid hormone (PTH) levels suggestive of hypoparathyroidism, and serum morning cortisol levels in the normal range-these features of mucocutaneous candidiasis and hypoparathyroidism contribute to the diagnosis of APS-1. The non-contrast Computed tomography (CT) scan of the brain revealed a unique finding: bilateral symmetrical calcifications, including subcortical white matter in the frontal, parietal, temporal lobes, and basal ganglia. He was treated with oral calcium, an antifungal oral suspension, and managed conservatively for dilated cardiomyopathy with beta-blockers, angiotensin receptor/neprilysin inhibitor (ARNi), and diuretics. At the same time, dysphagia and dysarthria settled after the resolution of candidiasis. This case of a rare disease brings knowledge about the discrete manifestations of APS type 1.