AN ATYPICAL PRESENTATION FOR PRIMARY SCLEROSING CHOLANGITIS

We describe the clinical course of a group of patients with primary sc lerosing cholangitis who at presentation were diagnosed to have autoim mune hepatitis. The history of one such patient is described in detail . We also compare this atypical sclerosing cholangitis (group I) to ty pical sclerosing cholangitis (group II) and to autoimmune hepatitis wi th (group III) and without (group IV) cholestasis. At presentation, me an AST in groups I and III was similar and significantly higher than i n group II (P < 0.05). Mean ALP was higher in sclerosing cholangitis t han in autoimmune hepatitis but not significantly so. Triaditis was pr esent in all patients in groups I, III, and IV. Piecemeal necrosis and multilobular collapse/fibrosis were equally frequent in groups I, III , and IV. Only the response to corticosteroids helped differentiate am ong groups. Groups III and IV responded by normalizing AST. In group I , AST improved, but never became normal. As ALP became disproportionat ely abnormal (ALP-predominant pattern), cholangiography was performed, and the diagnosis of primary sclerosing cholangitis was made in all g roup I patients. We recommend that cholangiography be performed early in patients with suspected autoimmune hepatitis who partially respond to corticosteroids and develop an ALP-predominant pattern.