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Introduction

The pathogenesis of autoimmune hepatitis is not known. It is often associated with other autoimmune diseases such as thyroiditis, lupus, rheumatoid arthritis, type I diabetes, celiac sprue, ulcerative colitis, vitiligo. Although autoimmune hepatitis is generally a chronic condition, it can also present acutely and is a rare cause of acute liver failure.

History

Personal or family history of autoimmune diseases such as thyroiditis, hyper or hypothyroidism, lupus, rheumatoid arthritis, vitiligo, etc

Physical examination

• Hepatomegaly/splenomegaly
• Rash
• Lymphadenopathy

In acute hepatocellular injury, the presence of asterixis (encephalopathy) and coagulopathy defines acute liver failure. Patients should be urgently referred to liver specialists.

Patients with evidence of encephalopathy (confusion/asterixis) should be urgently referred to a liver specialist or a transplant center.

Laboratory investigation

In addition to the relevant investigations based on the clinical presentation, the following liver tests should be ordered:

• Antinuclear antibodies (ANA)
• Anti-smooth muscle antibodies (ASMA)
• Protein electrophoresis (hypergammaglobulinemia)
• Anti-LKM antibodies (if above investigations are negative and autoimmunity is still suspected)
• PT/INR

Patients who develop coagulopathy (prolonged PT/INR) should be urgently referred to a liver specialist or a transplant center.