Overlap syndromes among autoimmune liver diseases.
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 standardised 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.
Department of Gastroenterology and Hepatology, University of Amsterdam, Amsterdam NL-1100 DE, The Netherlands.
Christian
C
Rust
Ulrich
U
Beuers
D000293:Adolescent; D000328:Adult; D002648:Child; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid