Scand J Gastroenterol 1987 Aug;22(6):655-64

Primary sclerosing cholangitis: a long-term follow-up study

Aadland E, Schrumpf E, Fausa O, Elgjo K, Heilo A, Aakhus T, Gjone E
Medical Dept. A, Rikshospitalet, University of Oslo, Norway.

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