Autoimmune hepatitis (previously known as chronic active hepatitis) is characterized by chronic inflammation of the liver associated with circulating autoantibodies and elevated serum globulins.
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.
Codes
ICD10CM: K75.4 – Autoimmune hepatitis
SNOMEDCT: 408335007 – Autoimmune Hepatitis
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Differential Diagnosis & Pitfalls
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.
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.
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
