Autoimmune Polyglandular Syndrome Type II in Pregnancy: A Case Report of Successful Multidisciplinary Management

Abstract

Autoimmune polyglandular syndrome type II (APS-II), also known as Schmidt syndrome, is a rare autoimmune disorder characterized by the coexistence of Addison's disease with autoimmune thyroid disease and/or type 1 diabetes mellitus. Pregnancy in women with APS-II poses significant challenges as adrenal insufficiency, thyroid dysfunction, and diabetes may adversely affect both maternal and fetal outcomes. We report a case of a 32-year-old primigravida with known autoimmune polyglandular syndrome type II comprising autoimmune hypothyroidism and primary adrenal insufficiency (Addison's disease), who was successfully managed through pregnancy and delivery. The patient had been on thyroid hormone replacement since childhood but experienced an adrenal crisis following discontinuation of thyroid medication, which led to the diagnosis of APS-II. She was subsequently maintained on hydrocortisone, fludrocortisone, and levothyroxine replacement. She was admitted at 37 weeks and 4 days of gestation for planned delivery with close maternal and fetal monitoring. A multidisciplinary approach involving obstetrics and endocrinology teams was employed throughout the peripartum period. Stress-dose glucocorticoid coverage was administered during labour, and the patient had an uneventful normal vaginal delivery of a live term female infant weighing 2.736 kg with Apgar scores of 8/10 and 9/10. This case highlights that with meticulous multidisciplinary care, appropriate hormone replacement, and close surveillance, favourable pregnancy outcomes can be achieved in women with APS-II.