When autoimmune hepatitis has features of primary biliary cholangitis or primary sclerosing cholangitis, these mixed clinical phenotypes constitute overlap syndromes. Diagnostic criteria have been promulgated, but clinical judgement and liver tissue examination remain cornerstones of diagnosis. Cholestatic laboratory and histological findings, concurrent inflammatory bowel disease, or non-response to conventional corticosteroid therapy compel practitioners to consider overlap in patients with autoimmune hepatitis. Laboratory indices of marked liver inflammation and histological findings of moderate to severe interface hepatitis, especially with lymphoplasmacytic infiltration, also warrant consideration of overlap in patients with primary biliary cholangitis or primary sclerosing cholangitits. Treatment recommendations to date have been based on weak clinical evidence, and disease management should be individualized and guided by the predominant disease component. Prednisone or prednisolone in combination with azathioprine has been used in patients with predominantly autoimmune hepatitis, whereas low dose ursodeoxycholic acid in conjunction with corticosteroid-based regimens has been recommended in syndromes with predominately cholestatic disease. All treatments have been variably effective, especially in patients with overlapping features of primary sclerosing cholangitis. Mycophenolate mofetil and calcineurin inhibitors have been used as salvage therapies in limited experiences, and liver transplantation has been associated with graft and overall survivals similar to those of the classical unmixed diseases.
This review contains 6 figures, 7 tables and 50 references
Keywords: autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, overlap, cholestatic laboratory and histological features
A patient with primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis variant syndrome
