Autoimmune liver diseases are divided into autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). They play an important role in the differential diagnosis of acute and chronic liver diseases. Diagnostic criteria consist of biochemical parameters (liver function tests, immunoglobulins, autoantibodies), imaging (ultrasound, MRCP, ERCP) and histopathologic characteristics. The treatment of choice in AIH is a combined immunosuppressive therapy of prednisolone and azathioprine. Ursodeoxycholic acid is the treatment of choice for PBC and improves liver biochemistry and prolongs transplant-free survival by slowing histological progression in the majority of patients. To date, there is no effective medical treatment option for patients with PSC.
