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AIH is a chronic inflammation of the liver of unknown cause, associated with circulating autoantibodies and hyperglobulinemia.
- Women are affected more than men (gender ratio 3.6:1).
- Extrahepatic manifestations may be found in 30%–50% of patients and include synovitis, celiac disease, Coombs-positive hemolytic anemia, autoimmune thyroiditis, Graves’ disease, rheumatoid arthritis, ulcerative colitis (UC), and other immune-mediated processes.
Two types of AIH have been proposed based on differences in their immunologic markers. They have a good response to corticosteroid therapy.
- Type I AIH is the most common form of the disease and constitutes 80% of AIH cases. It is associated with antinuclear antibodies (ANAs) and anti–smooth muscle antibodies (SMAs).
- Type 2 AIH is characterized by antibodies to liver/kidney microsome type 1 (anti-LKM1) and/or liver cytosol type 1 (anti-LC1). This type is predominately seen in children and young adults.