An Atypical Presentation for Primary Sclerosing Cholangitis

Abstract

We describe the clinical course of a group ofpatients with primary sclerosing cholangitis who atpresentation were diagnosed to have autoimmunehepatitis. The history of one such patient is describedin detail. We also compare this atypicalsclerosing cholangitis (group I) to typical sclerosingcholangitis (group II) and to autoimmune hepatitis with(group III) and without (group IV) cholestasis. Atpresentation, mean AST in groups I and III was similar andsignificantly higher than in group II (P < 0.05).Mean ALP was higher in sclerosing cholangitis than inautoimmune hepatitis but not significantly so. Triaditis was present in all patients in groups I, III,and IV. Piecemeal necrosis and multilobularcollapse/fibrosis were equally frequent in groups I,III, and IV. Only the response to corticosteroids helpeddifferentiate among groups. Groups III and IV responded bynormalizing AST. In group I, AST improved, but neverbecame normal. As ALP became disproportionately abnormal(ALP-predominant pattern), cholangiography was performed, and the diagnosis of primarysclerosing cholangitis was made in all group I patients.We recommend that cholangiography be performed early inpatients with suspected autoimmune hepatitis who partially respond to corticosteroids anddevelop an ALP-predominant pattern.