Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis with unusual initial presentation as fulminant hepatic failure

• Published in: Digestive and liver disease 2004-06, Vol.36 (6), p.419-422
• Main Authors: Kayacetin, E, Köklü, S, Temuçin, T
• Format: Article
• Language: English
• Subjects: Cholagogues and Choleretics - therapeutic use; Female; Glucocorticoids - therapeutic use; Hepatitis, Autoimmune - complications; Hepatitis, Autoimmune - diagnosis; Hepatitis, Autoimmune - drug therapy; Humans; Liver Cirrhosis, Biliary - complications; Liver Cirrhosis, Biliary - diagnosis; Liver Cirrhosis, Biliary - drug therapy; Liver Failure - drug therapy; Liver Failure - etiology; Middle Aged; Prednisolone - therapeutic use; Ursodeoxycholic Acid - therapeutic use
• ISSN: 1590-8658
• DOI: 10.1016/S1590-8658(04)00087-8

Autoimmune hepatitis and primary biliary cirrhosis are generally easy to discriminate on the basis of clinical, laboratory, and histological findings. The presence of anti-mitocondrial antibodies seropositivity and cholestatic clinical, laboratory, and/or histological features in patients with autoimmune hepatitis indicates the overlap syndrome of autoimmune hepatitis and primary biliary cirrhosis. Fulminant hepatic failure is an unusual initial form of presentation of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. We report the case of a 50-year-old woman with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome who presented with fulminant hepatic failure. Fulminant hepatic failure has a high mortality rate and may require liver transplant. Our patient revealed a good response to corticosteroid and ursodeoxycholic acid therapy. It is important to identify and distinguish autoimmune hepatitis and variant syndromes from other forms of liver disease because of response to corticosteroid therapy.