title: Primary sclerosing cholangitis: clinical and immunopathological review of 21 cases.
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.
authors.affiliation: Department of Medicine, University of Western Australia, Perth.
authors.forename: G P
authors.initials: GP
authors.lastname: Jeffrey
authors.forename: W D
authors.initials: WD
authors.lastname: Reed
authors.forename: B H
authors.initials: BH
authors.lastname: Laurence
authors.forename: K B
authors.initials: KB
authors.lastname: Shilkin
mesh_terms: D001323:Autoantibodies / Q000032:analysis; D001652:Bile Ducts / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology; D015209:Cholangitis, Sclerosing / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D005260:Female; D015795:HLA-B8 Antigen / Q000032:analysis; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D008297:Male; D008875:Middle Aged; D016176:T-Lymphocyte Subsets / Q000166:cytology; D014904:Western Australia / Q000453:epidemiology
chemical_list: D001323:Autoantibodies; D015795:HLA-B8 Antigen; D007136:Immunoglobulins