Autoimmune hepatitis (AIH) is a chronic inflammatory liver disorder with an etiology that remains unclear. The pathogenesis may be a result of alterations in immune tolerance, a genetic predisposition and environmental conditions, which in collaboration induce a T-cell-mediated attack on liver antigens, leading to necro-inflammation and liver damage. AIH has marked female predilection. AIH can present at all ages, but the two peak ages of incidence are in childhood or adolescence and at around 40 years of age. Trademark biochemical/serological characteristics of AIH are elevated aminotransferase levels, positivity for autoantibodies and increased IgG. A histological picture of interface hepatitis is typical of AIH. The value of immunosuppressive therapy with glucocorticoids, alone or in combination with azathioprine, was well documented in numerous studies of AIH.
Patients seropositive for ANA and/or anti-smooth muscle autoantibodies (SMA) have AIH type-1 whereas those presenting with positivity for anti-liver kidney type-1 autoantibody (anti-LKM-1) or anti-liver cytosol type-1 (anti-LC-1) have AIH type-2.
