Autoimmune hepatitis

1. Big picture

Autoimmune hepatitis (AIH) is a chronic immune-mediated inflammatory liver disease. The exam pattern is:

Young or middle-aged woman
+ fatigue / jaundice / raised ALT and AST
+ high IgG / hypergammaglobulinemia
+ ANA or anti-smooth muscle antibody positivity
+ liver biopsy with interface hepatitis
+ good response to corticosteroids
= autoimmune hepatitis

The most important exam message: AIH is treatable, but untreated disease can progress to cirrhosis even in young patients. Liver biopsy is usually needed to establish the diagnosis, and treatment is based on corticosteroids plus steroid-sparing immunosuppression, classically azathioprine.