Dourakis SP, et al. Autoimmune hepatitis associated with the antiphospholipid syndrome. Eur J Gastroenterol Hepato 2001;13:591-3.
Al-Khalidi JA, et al. Curent concepts in the diagnosis, pathogenesis, and treatment of autoimmune hepatitis. Mayo Clin Proc 2001;76:1237-52.
Take home points:
1. Think of autoimmune hepatitis, when more common causes of liver disease have been ruled out.
2. Think of autoimmune hepatitis in young women with cirrhosis and hypergammaglobulinemia.
3. Antibody tests in autoimmune hepatitis include anti-smooth muscle antibody and anti-LKM antibody
4. Prognosis is generally good and treatment should be reserved for patients with active hepatitis and/or
• 70% of patients with classic (type 1) autoimmune hepatitis (AIH) are females under the age of 40
• However, AIH can affect patients of all ages and has a global distribution
• Associated with other autoimmune diseases, including (in rare cases) antiphospholipid antibody
Making the diagnosis:
• Before thinking of AIH, rule out other causes of chronic liver disease:
− Hereditary (Wilson’s, hemochromatosis, alpha-1-anti-trypsin deficiency)
− Viral (hepatitis A, B, C, etc)
− Drug-induced (minocycline, INH, hydralazine, nitrofurantoin, etc)
• A clue to the diagnosis is hypergammaglobulinemia (elevated total protein, decreased albumin, and
decreased anion gap).
• A cholestatic picture on LFTs in AIH is extremely rare and should lead you to consider other diagnoses.
• Antibodies can help delineate the type of autoimmune hepatitis.
− Type 1 AIH: anti-smooth muscle antibody; ANA also frequently positive.
− Type 2 AIH: anti-LKM antibody, and-LC1 antibody
− Type 3 AIH: anti-SLA/LP antibody
• Liver biopsy is gold standard for diagnosis, but both biopsy and antibodies can be abnormal in other liver
diseases (especially viral hepatitis).
Types of autoimmune hepatitis:
• Type 1 (classic) AIH: all ages, female predominance, caucasia