autoimmuunhepatitis

Autoimmune hepatitis, known in Dutch as autoimmuunhepatitis, is a chronic inflammatory liver disease in which the immune system damages hepatocytes. It may be asymptomatic or present with fatigue, jaundice, abdominal discomfort, and pruritus. Without treatment, it can progress to cirrhosis and liver failure.

AIH is divided into two main types. AIH-1, the more common form in adults, is associated with antinuclear antibodies (ANA) and/or smooth muscle antibodies (SMA/ASMA). AIH-2 occurs mainly in children and is associated with anti-LKM-1 antibodies. Atypical antibody combinations may occur.

The pathogenesis involves genetic susceptibility, particularly HLA-DR3 and HLA-DR4, and loss of tolerance with autoreactive T cells causing interface hepatitis. Triggers are not clearly defined.

Diagnosis relies on a combination of liver test patterns, elevated IgG, autoantibodies, and histology, with exclusion of viral hepatitis, drug-induced injury, and other causes. Liver biopsy typically shows interface hepatitis with a plasma cell-rich infiltrate.

Treatment uses immunosuppression, usually starting with corticosteroids (prednisone or prednisolone) and often combined with azathioprine. Many patients achieve lasting remission; some require long-term maintenance or alternative agents such as mycophenolate mofetil. Relapses after withdrawal are common.

Prognosis improves with treatment; early diagnosis and adherence to therapy reduce the risk of progression. The condition predominantly affects females and can present at any age.