Autoimmune Polyglandular Syndrome Type II

Abstract

Abstract

Autoimmune Polyglandular Syndrome Type 2 (APS-2), also called Schmidt’s syndrome, is a rare endocrine disorder characterized by primary adrenal insufficiency with autoimmune thyroid disease or type 1 diabetes mellitus. We report a case of a 40-year-old female patient with a history of primary hypothyroidism who presented to the emergency department with abdominal pain, vomiting, hypotension, hypoglycemia, and progressive hyperpigmentation. Laboratory studies confirmed an acute Addisonian crisis and revealed profound hyponatremia, hypocalcemia, hypomagnesemia, low serum cortisol, high adrenocorticotropic hormone (ACTH), and an elevated thyroid-stimulating hormone (TSH). The physiologic stress of an incidental hormonally inactive left ovarian serous cystadenoma likely precipitated this acute life-threatening event and also contributed to the unmasking of the patient’s underlying adrenal deficiency. She was successfully resuscitated with intravenous fluids, vasopressors, and corticosteroid replacement and was discharged on a long-term regimen of oral steroids and thyroxine. This case highlights the significance of a high clinical suspicion for concomitant endocrine failures in patients with known autoimmune endocrinopathies presenting with acute systemic deterioration, as an early multidisciplinary intervention is key to improving prognosis and avoiding death.