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Introduction

Autoimmune hepatitis (AIH) is a chronic autoimmune liver disease characterized by progressive hepatocellular injury that can ultimately lead to cirrhosis.  AIH predominantly affects women and has a bimodal age distribution, peaking in the 20s and 40s-50s.

Pathophysiology and histopathology

The pathogenesis of AIH may involve exposure to environmental triggers (eg, viruses, medications, vaccinations, herbs) in genetically susceptible patients (eg, those with a high-risk HLA allele), leading to regulatory T-cell dysfunction.  The immune dysfunction ultimately results in loss of self-tolerance and a T-cell–mediated immune attack against hepatocytes, causing hepatocellular damage.  Histopathologic examination reveals portal and periportal lymphoplasmacytic inflammation with damage to periportal hepatocytes (interface hepatitis) ( Image 1).

Clinical presentation

AIH presents heterogeneously, ranging from asymptomatic aminotransferase elevations (25%) to cirrhosis (33%) or fulminant hepatic failure.  Most commonly, patients have symptoms that include:

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