Abstract
Review of liver biopsy specimens, autopsy specimens and clinical records of 107 patients with chronic ulcerative colitis and hepatobiliary diseases showed pericholangitis (defined as small-duct primary sclerosing cholangitis) in 37 (35%), primary sclerosing cholangitis (defined as large-duct primary sclerosing cholangitis) in 18 (17%), chronic active hepatitis in 14 (13%), cryptogenic cirrhosis in 12 (11%) and miscellaneous lesions including malignancies in 26 (24%). Documented cirrhosis was present or developed in 37 patients (35%). The spectrum of histologic features of small-duct primary sclerosing cholangitis was indistinguishable from that of confirmed large-duct primary sclerosing cholangitis. In 6 of the 18 patients who eventually developed the large-duct disease, biopsy evidence 1-12 yr earlier had shown small-duct primary sclerosing cholangitis. Small-duct and large-duct primary sclerosing cholangitis seem to be components of a disease spectrum.