Autoimmune polyglandular syndrome type 2: A case report.

ABSTRACT

Introduction: Autoimmune polyglandular syndrome 2(APS 2) is immune-mediated destruction that affects two or more endocrine glands and causes a constellation of multiple glands insufficiencies. Case presentation we reported a rare case 9 years old male diagnosed with APS 2; he had adrenal insufficiency three years ago due to leak adherence to hydrocortisone. He was admitted to the hospital for adrenal crises after hemodynamic stability; laboratory evaluation showed that he had Hashimoto's thyroiditis, celiac disease, and the glutamic acid decarboxylase antibody (GAD) Anti-islet cell antibodies were positive, so he was also predisposed to DM 1 later. Discussion: APS 2, also known as Schmidt's syndrome, is usually defined by the occurrence of the same fludrocortisone or more of the followings primary adrenal insufficiency (Addison's disease), Grave's disease, primary hypothyroidism, type 1 diabetes mellitus, celiac disease, and pernicious anemia. Conclusion: This case report underlines the importance of early recognition and treatment of acute endocrine diseases and the necessity to investigate pediatric patients with autoimmune diseases for coexisting conditions.