Therapie der autoimmunen Lebererkrankungen

Since the 1950s, autoimmune hepatitis has been recognized as a chronic liver disease that responds to treatment with steroids. Nevertheless, even today 25% of all diagnosed patients still present with liver cirrhosis, which emphasizes the importance of a timely and efficient diagnosis. Steroid-containing immunosuppression is characterized by a high rate of unwanted side effects. For non-cirrhotic patients, the synthetic steroid budesonide, which is characterized by high first-pass metabolism, represents a study-evaluated alternative. In the new guidelines for the management of primary sclerosing cholangitis, the therapeutic role of ursodeoxycholic acid has been modified, with no other effective alternative drugs being available. Management of sclerosing cholangitis is also made more difficult by the high risk of cholangiocarcinoma and colon carcinoma in addition to new differential diagnoses such as secondary sclerosing cholangitis and IgG4-associated cholangitis. IgG4-associated cholangitis can mimic primary sclerosing cholangitis but--in contrast-- can respond to the administration of steroid-containing immunosuppression.