Ulcerative colitis and primary sclerosing cholangitis as part of autoimmune polyglandular syndrome type III.

To the Editor: Autoimmune polyglandular syndrome (APS) is an autoimmune disease affecting 2 or more endocrine organs; generally 4 types of APS are distinguished. APS type III as a subdivision of type II is characterized by autoimmune thyroid disease with other autoimmune disorders but without adrenal insufficiency.1 We report a case of a 37-year-old man initially diagnosed with ulcerative colitis (UC) who was found to have APS type III. In a 10-year period different clinical, laboratory, and radiological examinations revealed the presence of coexisting UC, primary sclerosing cholangitis (PSC), insulin-dependent diabetes mellitus (IDDM), Hashimoto’s thyroiditis, vitiligo, and rheumatoid arthritis (RA). The rare combination of these different, severe syndromes has not been reported before. Inflammatory bowel diseases are unusual as part of APS; however, celiac disease is the most commonly associated, immune-mediated enteral disease in APS type III.2 The presence of UC in any type of APS has not been documented before. The medical history of this 37year-old man started in 1994 when he was diagnosed with UC pancolitis accompanied by PSC involving both the extraand intrahepatic bile ducts. IDDM developed 2 years after the diagnosis of UC and PSC. After a long-lasting period with chronic mild activity, he had a flare-up of UC with 5–10 bloody stools daily in 2006. Control endocrine examinations performed in May 2007 revealed an elevated thyroid-stimulating hormone level of 199.5 mIU/L and an abnormal anti-thyroid peroxidase antibody level of 311.7 iu/mL, indicating Hashimoto’s thyroiditis. Therefore, Lthyroxin treatment was administered. In October 2007, because of the continuous endoscopic and clinical activity of UC despite the usual mesalazine and azathioprine treatment, cyclosporine was introduced but had to be discontinued because of the development of weakness and myalgia. In the same year, patchy vitiligo was observed affecting the whole body. In March 2008 the presence of a deforming polyarthritis involving both wrists, elbows, knees, and the small joints of the hands and feet was noted. The diagnosis of RA was established based on symmetric polyarthritis and the typical radiographic changes, including multiple juxta-articular erosions in the metacarpo-phalangeal, proximal interphalangeal, intercarpal, and radiocarpal joints and multiple ankyloses in the intercarpal joints (Fig. 1). IgM rheumatoid factor and antimutated citrullinated vimentin antibodies were negative; dehydroepiandrosterone sulfate, adrenocorticotropic hormone, and cortisol test were also within the normal range. Coexistence of UC, PSC, Hashimoto’s thyroiditis, IDDM, vitiligo, and RA indicated the diagnosis of APS type III. Gluten sensitive enteropathy was excluded by serological examinations and duodenal biopsy. Copyright © 2009 Crohn’s & Colitis Foundation of America, Inc. DOI 10.1002/ibd.20913 Published online 25 March 2009 in Wiley InterScience (www.interscience.wiley.com). FIGURE 1. Vitiligo and rheumatoid arthritis with multiple erosions and ankyloses on the radiographic finding. [Color figure can be viewed in the online issue, which is available at www.interscience.wiley.com]