Autoantibodies and Autoantigens in Autoimmune Hepatitis

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

Disclosures

Semin Liver Dis. 2002;22(4) 

In This Article

Autoimmune Hepatitis Type 1

When AIH is classified according to the presence of serological markers, ANA characterize AIH type 1. In most cases, SMA are also present. The clinical profile of ANA-positive AIH shows hypergammaglobulinemia in 97% of patients with an elevated immunoglobulin G. This form of AIH represents 80% of all cases of AIH, making it the most prevalent subclass. ANA-positive AIH was first described as lupoid, classical, or idiopathic AIH by Mackay.[5] Seventy percent of patients are women, with a peak incidence between ages 16 and 30 years. It is noteworthy that 50% are older than 30 years. An association with other immune syndromes is observed in 48%, with autoimmune thyroid disease, synovitis, and ulcerative colitis as leading associations. The clinical course is usually not fulminant or clinically spectacular, and an acute onset is very rare. However, in rare cases AIH may start as fulminant hepatitis. About 25% have cirrhosis at the time of diagnosis.

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