Overlap syndromes.
In hepatology, the term overlap syndrome describes variant forms of the major hepatobiliary autoimmune diseases, autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). Patients with overlap syndromes present with both hepatitic and cholestatic biochemical and histological features of AIH, PBC, and/or PSC, and usually show a progressive course toward liver cirrhosis and liver failure without adequate treatment. AIH-PBC overlap syndromes have been reported in almost 10% of adults with AIH or PBC, whereas AIH-PSC overlap syndromes were found in 6 to 8% of children, adolescents, and young adults with AIH or PSC. A minority of patients may also show transition from stable PBC to AIH, AIH to PBC, or AIH to PSC, as documented by single case reports and small case series. Single cases of AIH and autoimmune cholangitis (antimitochondrial antibody-negative PBC) overlap have also been reported. Empiric medical treatment of AIH-PBC and AIH-PSC overlap syndromes includes anticholestatic therapy with ursodeoxycholic acid and immunosuppressive therapy with corticosteroids and azathioprine. In end-stage disease, liver transplantation is the treatment of choice.
Beuers
Ulrich
U
Department of Medicine II-Grosshadern, Ludwig Maximilians-University of Munich, Germany. Beuers@med.uni-muenchen.de
Rust
Christian
C
D000328:Adult; D017668:Age of Onset; D000368:Aged; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D015897:Comorbidity; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D017093:Liver Failure / Q000209:etiology; D008297:Male; D011379:Prognosis; D013577:Syndrome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid