Characteristics of autoimmune hepatitis in patients who are not of European Caucasoid ethnic origin

Background: Significant diversity in disease severity has been identified for autoimmune disorders among different ethnic groups but there is a lack of data on autoimmune hepatitis (AIH) in populations other than those of European Caucasoid (EC) or Japanese extraction. Aims: To assess the clinical features, response to therapy, and eventual outcome in AIH patients of non-EC ethnicity. Methods: A retrospective review of a regularly updated database of patients with AIH referred to liver outpatient clinics at King's College Hospital, London, since 1983. Results: Twelve patients were identified (10 female; six African, five Asian, one Arabic; median age at presentation 30 years (range 12-58)) who satisfied international criteria for type 1 (1 1 cases) or type 2 (one case) AIH. Nine (75%) had cholestatic serum biochemistry and three (25%) had mild biliary changes on liver biopsy without definitive features of primary biliary cirrhosis or cholangiographic evidence of primary sclerosing cholongitis. Four showed a complete biochemical response to standard prednisolone with or without azathioprine therapy, three partial, and five no response. Four have required liver transplantation for intractable disease. By comparison with 180 EC patients with definite AIH attending during the same period, the non-EC patients were younger (p<0.05), presented with cholestatic biochemistry (p=0.014), and morphological biliary features more frequently (p<0.0005) and showed a poorer initial response to standard therapy (p<0.0005). Conclusions: Clinical expression of AIH in non-EC patients seems to differ in important respects from that in EC or Japanese patients. Management of such patients is challenging and may require alternative or more aggressive treatment strategies.