During the 10-year period from 1 January 1975 to 31 December 1984, primary sclerosing cholangitis (PSC) was diagnosed in 45 patients. Twelve of the patients have died (26.7%), 10 of them of causes related to PSC. Inflammatory bowel disease was found in all patients; ulcerative colitis was found in 37, Crohn's disease in 6, and unclassified colitis in 2 patients. Of the patients alive, 27 were submitted to a follow-up study in 1985. At the follow-up examination no general progression of the liver disease, as measured on the basis of clinical examination and levels of transaminases, alkaline phosphatases, and bilirubin, was found. Cholangiographic evaluation in 24 patients showed that the stage of ductal changes progressed from mild to moderate in 3 patients; in the other patients the stage was not altered. Morphologic examination of liver biopsy specimens in patients with a benign clinical course usually showed portal inflammation, fibrosis, and minor signs of piecemeal necrosis, whereas widespread piecemeal necrosis was found in patients who deteriorated and died. The 50% survival since diagnosis of liver disease was calculated to be 17 years in patients with PSC and 50 years in a comparable group among the general population. The estimated survival curve in PSC was displaced to the left, indicating a reduced life expectancy of about 30 years.
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.
In a 5-year period 48 (14%) of 336 patients with ulcerative colitis were found to have hepatobiliary disease. The bile ducts were examined in 35 of these patients, and optimal visualization of both intra- and extra-hepatic bile ducts was obtained in 26. Duct changes compatible with sclerosing cholangitis were found in 14 patients. This finding of sclerosing cholangitis in 4% of all patients admitted with ulcerative colitis by far exceeds previous estimations on the incidence of sclerosing cholangitis in ulcerative colitis. The entire colon was usually affected, and the symptoms of the bowel disease were most often mild or moderate. The age at the onset of the colitis was usually below 20 years in patients with combined ulcerative colitis and hepatobiliary disease. In most patients the hepatobiliary disease gave no symptoms. Biochemical data and the histological findings in the liver biopsies did not distinguish between patients with hepatobiliary disease with and without sclerosing cholangitis. Our follow-up study has so far shown that most patients with sclerosing cholangitis remain asymptomatic for a considerable period of time.
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