Beyond Adrenal Crisis: Protean Manifestations of Hypocalcemia in Autoimmune Polyglandular Syndrome Type 1

Autoimmune polyglandular syndrome type 1 (APS1) is associated with adrenal insufficiency, hypoparathyroidism, mucocutaneous candidiasis, and primary ovarian insufficiency. Our objective is to describe 2 acute presentations unrelated to adrenal crisis in a patient with APS1: 1) stress-induced cardiomyopathy presenting as shock and heart failure and 2) acute on chronic urinary retention.

A 40-year-old patient with APS1 was admitted to the intensive care unit with cardiogenic shock. She was diagnosed with nonischemic cardiomyopathy with a left ventricular ejection fraction of 20% to 25% in the setting of severe hypocalcemia. Her heart function recovered after 1 year, and the working diagnosis was stress-induced cardiomyopathy complicated by hypocalcemia.At 50 years old, she presented to the emergency department with abdominal pain and tingling in her hands and feet. She was found to have severe hypocalcemia as well as acute on chronic urinary retention. After intravenous calcium supplementation, her symptoms subsided, and she was able to void once again and was discharged home without urinary catheter. Subsequent urological studies demonstrated detrusor overactivity and severe pelvic floor dysfunction.

Acute hypocalcemia is known to cause neuromuscular irritability, and there is increasing evidence that this can also precipitate stress-induced cardiomyopathy and urinary retention. Female APS1 patients are further predisposed to both conditions due to hypoestrogenism from primary ovarian failure.

Atypical sequelae of hypoparathyroidism-associated hypocalcemia, such as cardiomyopathy and urinary retention, warrant consideration in acute presentations of patients with APS1.