Primary Sclerosing Cholangitis: A Long-Term Follow-up Study

Abstract

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.

The Child-Pugh classification as a prognostic indicator for survival in primary sclerosing cholangitis

“…44,45 In and 9 years. 41 Two notable exceptions to this uniformly dis-our population, the CPC was found to effectively discriminate mal outlook for patients with PSC are the studies published between survival amongst the three classes in a statistically by Helzberg et al, 26 and by Aadland et al 42 The former group significant manner; the DSM did not provide additional prereported that only 19% of their patients died during an aver-dictive ability to the CPC. age follow-up of 56 months with an estimated survival of 75%…”

Primary sclerosing cholangitis

“…The median reported survival in patients with PSC is 10 to 12 years from the time of diagnosis, 42,74,75 although an active search for PSC in screening programs may lead to the identification of patients in an early stage of disease who can expect a significantly longer survival. 47,76 The disease, however, generally follows a progressive course, and most asymptomatic patients eventually develop symptoms of chronic cholestasis and biliary cirrhosis with its consequent complications. 2 Several variables such as older age; serum levels of bilirubin, albumin, and aspartate aminotransferase; hepatosplenomegaly; variceal bleeding; presence of IBD; and histological stage have been identified as independent risk factors for predicting a poor prognosis.…”

“…[41][42][43][44][45] The autopsy prevalence is much higher with cholangiocarcinoma, it being reported in 30% to 42% of patients with PSC undergoing autopsy. [45][46][47][48] Cholangiocarcinoma may develop in one third of patients with PSC if follow-up is extended long enough. 29 A large clinical experience has reported an annual incidence of less than 1%.…”

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

“…Primary sclerosing cholangitis (PSC) is an idiopathic, fibro‐inflammatory, cholestatic liver disease that can affect both the intrahepatic and extrahepatic biliary ductal systems 1, 2, 3. PSC follows a progressive course that can culminate in cirrhosis, portal hypertension, liver failure, and/or cholangiocarcinoma (CCA).…”