Cholestatic liver disease overlap syndromes

Primary biliary cirrhosis and primary sclerosing cholangitis share some clinical features with autoimmune hepatitis, but when features of autoimmune hepatitis are present, prognosis can be affected and immunosuppressive treatment warranted. The presence of severe interface hepatitis in primary biliary cirrhosis portends a worse prognosis and should prompt evaluation for possible autoimmune hepatitis overlap and treatment with immunosuppression. Specific models to identify which subjects benefit most from the addition of immunosuppression need to be developed. Drug-induced liver injury and IgG4 disease may masquerade as autoimmune hepatitis or primary sclerosing cholangitis and are important to consider in the differential diagnosis of the overlap or variant syndromes.