Autoimmune Overlap Syndromes

  • Philippe J. ZamorEmail author


Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are autoimmune diseases of the liver. AIH has two major variants in which the features of classic AIH can coexist with either PBC, (formerly known as primary biliary cirrhosis) and PSC. AIH-PBC overlap syndrome (OS) occurs far more commonly than AIH-PSC OS. The recognition of these overlap syndromes mainly comes into significance because of lack of response to conventional corticosteroid therapy for AIH. Careful use of clinical and histologic criteria may be necessary for diagnosis. The overlap syndromes can progress to cirrhosis and liver failure if not recognized and adequately treated. Standard guidelines for treatment of these overlap syndromes are lacking, and is considered empiric and extrapolated from data derived from the primary autoimmune liver diseases.


Overlap syndrome Primary biliary cholangitis Primary sclerosing cholangitis Cholangiogram AMA 


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Division of HepatologyCarolinas Medical Center-Atrium HealthCharlotteUSA

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