Autoimmune polyglandular syndrome type 2: A case report
Agyad Bakkour, Mohammed Deeb Zakkor, Lina Taha Khairy, Rostom Horo, Eman Mohammed sharif Ahmed, Hachem Alhussein
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. HIGHLIGHTS Autoimmune polyglandular syndrome 2(APS 2) is an extremely rare condition and an immune-mediated destruction, which is consider the first reported case from Syria in the literature. APS 2 is defined by the occurrence in the same time two or more of the followings: primary adrenal insufficiency (Addison's disease), Grave's disease, primary hypothyroidism, type 1 diabetes mellitus,celiac disease and pernicious anaemia. Follow-up is very necessary by evaluating the body mass index to detect the presence of weight loss and assessing the bone age of the wrist for growth.