Type II polyglandular autoimmune syndrome Print Images (1) Other Resources UpToDate PubMed Alerts and Notices Synopsis An autoimmune polyendocrinopathy with Addison disease as its main characteristic, in conjunction with autoimmune thyroid diseases and/or diabetes mellitus type 1. Associated autoimmune thyroid diseases include Hashimoto thyroiditis and Graves disease. Other conditions that may appear include hypothyroidism, hyperthyroidism, myasthenia gravis, hypogonadism, and celiac disease. Vitiligo may appear in up to 50% of cases. Etiology is not well understood, although some cases of autosomal-dominant inheritance have been observed. The syndrome is 3 times more common in females. Onset is in adulthood, most commonly around age 30. Treatment includes hormone replacement. Codes ICD10CM: E31.0 – Autoimmune polyglandular failure SNOMEDCT: 83728000 – Polyglandular autoimmune syndrome, type 2 Best Tests Subscription Required References Subscription Required Last Updated:01/25/2022 Copyright © 2023 VisualDx®. All rights reserved. Type II polyglandular autoimmune syndrome Print Copyright © 2023 VisualDx®. All rights reserved.