Autoimmune hepatitis

Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease rapidly leading to cirrhosis in untreated patients. Diagnosis of AIH can be challenging. Rational step wise diagnostic work-up is necessary as only a minority of patients with pathological liver function tests will suffer from AIH. Early diagnosis on the other hand is essential as immunosuppressive therapy leads to a good prognosis in these patients. Remission is almost universally achieved on steroids, and maintained with azathioprine, which should be given for more than three years in all patients. Careful evaluation before stop of immunosuppressive therapy is needed as early termination will lead to relapse of the disease. Only a minority of patients do not tolerate treatment or show insufficient response to standard therapy with steroids and azathioprine. For these patients there are several alternative treatment options though none of them is well established. Liver transplantation can normally be avoided by adequate treatment, but, it may be required in exceptional cases, such as fulminant disease or patients presenting with advanced decompensated cirrhosis.