Hepatic overlap syndromes

Hepatic overlap syndromes represent variant forms of the classical autoimmune hepatopathies, autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). They are ill-defined and present with both hepatitic and cholestatic biochemical and histological features of AIH, PBC, and/or PSC. Overlap syndromes mostly show a progressive course leading to end stage liver disease. AIH-PBC overlap syndromes have been described in nearly 10% of patients with AIH or PBC. AIH-PSC overlap syndromes may represent 6-8 % of patients with AIH or PSC mainly diagnosed in children, adolescents and young adults. Whether true overlap between PBC and PSC exists remains elusive. Autoimmune cholangitis (AIC) shares many features with PBC and is regarded as AMA-negative PBC, thus an outlier syndrome rather than an overlap syndrome. Single cases of AIH-AIC overlap have been reported. Coexistence of AIH and chronic hepatitis C does not justify use of the term overlap syndrome" for these cases. Medical treatment of AIH-PBC and AIH-PSC overlap syndromes is empiric and includes anticholestatic therapy with ursodeoxycholic acid (UDCA) of the cholestatic component and immunosuppressive therapy with corticosteroids and azathioprine of the hepatitic component of these disorders. Liver transplantation is the treatment of choice for end stage disease. © 2004 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved."