Multiple Disease Associations in Autoimmune Polyglandular Syndrome Type II

Autor: Boris Draznin, Amita Maturu, Aaron W. Michels
Rok vydání: 2014
Předmět:
Zdroj: Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists. 20(12)
ISSN: 1530-891X
Popis: Objective Autoimmune polyglandular syndrome type II (APS II) is characterized by adrenal insufficiency (Addison’s disease), autoimmune thyroid disease, and/or type 1 diabetes mellitus (DM1). Multiple other autoimmune diseases have been associated with APS II. Here we report a case of a patient with APS II who over the course of 10 years developed Addison’s disease, hypothyroidism, DM1, Hashimoto’s encephalopathy, vitiligo, celiac disease, seronegative arthritis, and ulcerative colitis. This is a particularly aggressive course of APS II, and this combination of autoimmune diseases has not been previously reported. Methods A 25-year-old female with a history of ulcerative colitis (UC), celiac disease, and DM1 presented to our institution with mental status changes. She was diagnosed with Hashimoto’s encephalopathy and treated with high-dose steroids and intravenous immunoglobulin (IVIG). She recovered well from her encephalopathy but her posthospitalization course was complicated due to the development of Addison’s disease, vitiligo, seronegative arthritis, and hypothyroidism. Results The current understanding of APS II and its autoimmune disease associations are briefly summarized. Submitted for publication April 10, 2014 Accepted for publication July 10, 2014 The association of UC and Hashimoto’s encephalopathy with APS II is novel and discussed in detail. Conclusion A case of a patient with APS II with a dramatic development of 8 autoimmune diseases over 10 years is described. The novel APS II developments of Hashimoto’s encephalopathy and UC are discussed. This case highlights the potential complexity and severity of the clinical course of APS II. (Endocr Pract. 2014;20:e250-e255)
Databáze: OpenAIRE