The etiology of this disease is not known. The International Autoimmune Hepatitis Group has published diagnostic criteria for autoimmune hepatitis that combines liver histology with serum laboratory studies. Other causes of chronic liver disease should be excluded.
The clinical manifestations of this disease vary widely. A subset of patients may be asymptomatic and may be identified by abnormal liver transaminases. Other patients will present with signs and symptoms of liver disease or cirrhosis. Patients may have fatigue, pruritus, nausea, or abdominal pain. Patients may have arthralgia involving the small joints. Hepatosplenomegaly and jaundice may or may not be present.
This disease is more common in women. Some population differences in presentation have been noted, but are not present in all cases. Individuals of African descent are more likely to present with cirrhosis. Patients of Asian descent are more likely to have mild disease. South American pediatric patients often have severe liver inflammation on presentation.
Other autoimmune conditions may be present concomitantly including thyroiditis, ulcerative colitis, type 1 diabetes mellitus, rheumatoid arthritis, and celiac disease.
In addition to elevated transaminases, laboratory evaluation may show increased bilirubin and alkaline phosphatase. Serum globulins (usually IgG) are usually elevated. Some combination of autoantibodies (eg, antinuclear antibodies [ANA], antismooth muscle antibodies [ASMA], anti-liver kidney microsomal antibodies [anti-LKM-1], or anti-liver cytosol antibodies [anti-LC1]) are present.
Type 1, or "classical," autoimmune hepatitis is characterized by +ANA and +ASMA antibodies.
Type 2 autoimmune hepatitis is characterized by +anti-LKM-1 or +anti-LC1 antibodies.
K75.4 – Autoimmune hepatitis
408335007 – Autoimmune Hepatitis
- Chronic hepatitis B – Screening testing should be performed.
- Chronic hepatitis C – Screening serology should be obtained.
- Cholangiopathy due to human immunodeficiency virus (HIV) – Screening test should be performed.
- Primary biliary cirrhosis – Patients have elevated alkaline phosphatase, antimitochondrial antibodies, and no extrahepatic biliary obstruction.
- Primary sclerosing cholangitis – Magnetic resonance cholangiography can be considered.
- Systemic lupus erythematosus (SLE) – Some patients with SLE have liver enzyme abnormalities, and some patients with autoimmune hepatitis (type 1) have positive ANA testing.
- Celiac disease – Serologic evaluation (IgA anti-TTG) may be performed.
- Alcoholic liver disease
- Nonalcoholic steatohepatitis
- Wilson disease – Serum ceruloplasmin level could be obtained.
- Drug-induced hepatotoxicity – Detailed medication history should be performed.
- Hemochromatosis – Iron studies with transferrin saturation can be checked.
- Alpha-1-antitrypsin deficiency – Serum levels of alpha-1-antithrypsin can be checked.