Primary sclerosing cholangitis: Clinical and immunopathological review of 21 cases
Article first published online: 28 JUN 2008
Journal of Gastroenterology and Hepatology
Volume 5, Issue 2, pages 135–140, March 1990
How to Cite
JEFFREY, G. P., REED, W. D., LAURENCE, B. H. and SHILKIN, K. B. (1990), Primary sclerosing cholangitis: Clinical and immunopathological review of 21 cases. Journal of Gastroenterology and Hepatology, 5: 135–140. doi: 10.1111/j.1440-1746.1990.tb01818.x
- Issue published online: 28 JUN 2008
- Article first published online: 28 JUN 2008
- Accepted for publication 29 May 1989.
- sclerosing cholangitis;
- T lymphocytes
Abstract Twenty-one patients with primary sclerosing cholangitis were seen during 1979–87. The mean age at onset of disease was 51.7 years (range: 13–78 years) with a male: female ratio of 2.5:1. Six (29%) were asymptomatic at the time of diagnosis. Eleven patients (52%) had ulcerative colitis. Cholangiography demonstrated abnormalities limited to the intrahepatic ducts in 10 cases, with both intrahepatic and extrahepatic involvement in 11. Histological features on liver biopsy included: portal tract inflammation and cholestasis in all; paucity of bile ducts in 56%; piecemeal necrosis in 19% and cirrhosis in 6%. Circulating autoantibodies and elevated serum immunoglobulins were found in half of the patients and HLA-B8 was detected in 53%. A deficiency of circulating CD3 and CD8 cells was not found in the 12 patients tested. The mean follow-up was 51 months (range: 3–180 months). Three patients died from non-hepatic causes and another has received liver transplantation. A Kaplan-Meier curve predicted 70% survival at 72 months.