Am J Gastroenterol 1998 May;93(5):777-84

Autoimmune hepatitis overlapping with primary sclerosing cholangitis in five
cases.

McNair AN, Moloney M, Portmann BC, Williams R, McFarlane IG

Institute of Liver Studies, King's College Hospital, London, United Kingdom.

Abstract
OBJECTIVE: We report five cases (four male; median age 20 yr, range 14-38 yr) of
an autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. The
patients presented with jaundice, elevated serum aminotransferase and alkaline
phosphatase activities, hyperglobulinemia with high immunoglobulin G (IgG)
levels, circulating antinuclear and/or smooth muscle autoantibodies (> or =
1:40), and moderate to severe interface hepatitis on liver biopsy (with biliary
features in four). METHODS: All five fulfilled criteria for diagnosis of
"definite" autoimmune hepatitis and showed marked responses to prednisolone and
azathioprine therapy, with relapses occurring during reduction or withdrawal of
treatment. Cholangiographic features of primary sclerosing cholangitis were
found in three patients at presentation and after intervals of 7 and 14 yr in
the other two. Only two had evidence of inflammatory bowel disease. Diagnostic
criteria for identifying those patients who may benefit from immunosuppressive
therapy were reviewed. RESULTS: Review of the literature revealed only 11
similar cases that were sufficiently well described for comparison. However, in
contrast to these and the present cases, preliminary data from other studies
have suggested a marked association with ulcerative colitis and a poor response
to immunosuppressive therapy. CONCLUSIONS: It is recommended that the
possibility of an autoimmune hepatitis/primary sclerosing cholangitis overlap
syndrome responsive to immunosuppressive therapy should be considered in any
patient presenting with a hepatitic illness with hyperglobulinemia, antinuclear
or smooth muscle autoantibodies, and biliary changes on liver biopsy.
Cholangiography should be considered in such patient