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Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Jun 7, 2008; 14(21): 3368-3373
Published online Jun 7, 2008. doi: 10.3748/wjg.14.3368
Overlap syndromes among autoimmune liver diseases
Christian Rust, Ulrich Beuers
Christian Rust, Department of Medicine II, Klinikum Grosshadern, University of Munich, Munich 81377, Germany
Ulrich Beuers, Department of Gastroenterology & Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam NL-1100 DE, The Netherlands
Correspondence to: Ulrich Beuers, MD, Professor of Gastroenterology & Hepatology, Department of Gastroentero-logy & Hepatology, Academic Medical Center, University of Amsterdam, PO Box 22700, Amsterdam NL-1100 DE, The Netherlands. u.h.beuers@amc.uva.nl
Telephone: +31-20-5662422
Fax: +31-20-6917033
Received: September 12, 2007
Revised: March 28, 2008
Accepted: April 4, 2008
Published online: June 7, 2008
Abstract

The three major immune disorders of the liver are autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). Variant forms of these diseases are generally called overlap syndromes, although there has been no standardized definition. Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC. The AIH-PBC overlap syndrome is the most common form, affecting almost 10% of adults with AIH or PBC. Single cases of AIH and autoimmune cholangitis (AMA-negative PBC) overlap syndrome have also been reported. The AIH-PSC overlap syndrome is predominantly found in children, adolescents and young adults with AIH or PSC. Interestingly, transitions from one autoimmune to another have also been reported in a minority of patients, especially transitions from PBC to AIH-PBC overlap syndrome. Overlap syndromes show a progressive course towards liver cirrhosis and liver failure without treatment. Therapy for overlap syndromes is empiric, since controlled trials are not available in these rare disorders. Anticholestatic therapy with ursodeoxycholic acid is usually combined with immunosuppressive therapy with corticosteroids and/or azathioprine in both AIH-PBC and AIH-PSC overlap syndromes. In end-stage disease, liver transplantation is the treatment of choice.

Keywords: Autoimmune hepatitis, Immunosuppressive agents, Primary biliary cirrhosis, Primary sclerosing cholangitis, Ursodeoxycholic acid