M. Kay et al., ASSOCIATED ULCERATIVE-COLITIS, SCLEROSING CHOLANGITIS, AND INSULIN-DEPENDENT DIABETES-MELLITUS, Cleveland Clinic journal of medicine, 60(6), 1993, pp. 473-478
We report two young men with clinical and laboratory evidence of macro
scopic utcerative colitis, sclerosing cholangitis, and insulin-depende
nt diabetes mellitus. The first patient presented at age 15 with vomit
ing, abdominal pain, weight loss, and abnormal liver function test res
ults. Liver biopsy and endoscopic retrograde cholangiopancreatography
(ERCP) demonstrated sclerosing cholangitis. Colonoscopy with biopsy re
vealed ulcerative colitis which responded to sulfasalazine. Diabetes o
ccurred at age 18 and insulin therapy was begun. The second patient wa
s 19 at presentation with diarrhea, hematochezia, and weight loss. Pro
ctosigmoidoscopy revealed ulcerative colitis, and sulfasalazine led to
clinical remission. Three months later he developed diabetes requirin
g insulin therapy. At age 28, he developed elevated alkaline phosphata
se, and ERCP revealed sclerosing cholangitis. At age 37 he expired fro
m adenocarcinoma that metastasized to the liver. Literature review rev
ealed only one possible case report of this association with microscop
ic asymptomatic ulcerative colitis in that patient. Statistical analys
is suggests that this association is real rather than a chance occurre
nce. An autoimmune process may be involved and a specific histocompati
bility locus antigen (HLA) type may exert a regulatory influence.