Pericholangitis in chronic ulcerative colitis: primary sclerosing cholangitis of the small bile ducts?

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 to 12 years earlier had shown small-duct primary sclerosing cholangitis. Thus, small-duct and large-duct primary sclerosing cholangitis seem to be components of a disease spectrum.