Sclerosing Cholangitis in Ulcerative Colitis

Abstract

In the 5-year period 1974-78, 48 (14%) of 336 patients with ulcerative colitis were found to have hepatobiliary disease. Endoscopic retrograde cholangiography (ERC) was successfully performed in 39 of these 48 patients, and sclerosing cholangitis was demonstrated in 19. One is excluded from this series because Crohn's disease was diagnosed at reclassification of the bowel disease. Two of the 18 patients with ulcerative colitis and sclerosing cholangitis have died, one of cholangiocarcinoma and one of an unrelated cause. The remaining 16 patients have been observed for a median period of 6 years (3-13 years) since the diagnosis of hepatobiliary disease. Ten have remained symptom-free, four have had intermittent or non-progressive symptoms, and two have developed symptoms of advanced chronic liver disease. The bilirubin level, which was initially raised in one of the patients, was elevated in four at the follow-up examination. Otherwise the laboratory values have remained stationary. Evidence of a progression of the hepatobiliary disease was found in most patients by repeated liver biopsy and particularly ERC. It is concluded that sclerosing cholangitis may remain asymptomatic for several years. Since progressive cholangiographic changes were often seen without concomitant worsening of symptoms, laboratory data, and liver biopsy findings, it is concluded that these criteria are of limited use in evaluating the progression of this disease.

Concomitant colitis associated with primary sclerosing cholangitis

“…Interestingly, approximately 80% of patients with PSC have UC in Western countries, [8][9][10] while only 2% to 5% of patients with UC have PSC. 8,[11][12][13] There is a very close relationship of etiology between PSC and UC.…”

Primary sclerosing cholangitis

“…There is a strong association between PSC and inflammatory bowel disease, particularly ulcerative colitis [3,4]. Approximately 4% of patients with inflammatory bowel disease either have or will develop PSC [5].…”

Outcome of Patients Undergoing Liver Transplantation for Primary Sclerosing Cholangitis

“…Epidemiologic studies have shown that approximately 70 percent of all patients with PSC have coexisting inflammatory bowel disease (IBD). 1,2 In addition, PSC occurs in 2 to 7 percent of patients with ulcerative colitis (UC) [3][4][5][6] and in approximately 1 to 3 percent of patients with Crohn's disease. 7 Patients with PSC have a substantially reduced survival, [8][9][10] caused by progressive fibrosis of the intrahepatic and/or extrahepatic bile ducts, which leads to cirrhosis and eventual liver failure or to the development of cholangiocarcinoma.…”