Autoantibodies and Autoantigens in Autoimmune Hepatitis

Christian P. Strassburg, MD, Michael P. Manns, MD


Semin Liver Dis. 2002;22(4) 

In This Article

Microsomal Autoantibodies in Autoimmune Hepatitis Associated With the Autoimmune Polyglandular Syndrome

APS-1 is a syndrome characterized by immune-mediated destruction of endocrine tissues, chronic candidiasis, and additional ectodermal disorders.[108,109] In about 10 to 18%, it is associated with AIH. It appears to be caused by mutations in a recently identified gene, the AIRE.[110] Similar to AIH type 2, hepatitis in APS-1 is associated with autoantibodies directed against CYP proteins. In patients with APS-1/APECED, autoantibodies to CYP 1A2 and CYP 2A6 have been described as markers of an autoimmune liver disease, but only antibodies to CYP 1A2 are specific for hepatitis in APECED, albeit with a low sensitivity.[22,23,24,25] Autoantibodies against molecular targets of microsomal autoantibodies typical for AIH type 2 have not been found in APECED patients.[23]


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