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Autoimmunerkrankungen der Leber

Autoimmune liver diseases

  • Schwerpunkt: Generalisierte und organbezogene Autoimmunerkrankungen
  • Published:
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Zusammenfassung

Die autoimmune Hepatitis (AIH) tritt in allen Altersgruppen auf und betrifft häufiger Frauen als Männer (Verhältnis 3:1). Einige Patienten können komplett asymptomatisch sein, andere zeigen eine akute Hepatitis bis hin zum Leberversagen. Histologisch ist die AIH durch ein lymphoplasmazelluläres Infiltrat gekennzeichnet. Biochemisch und serologisch finden sich erhöhte Leberenzyme, eine Hypergammaglobulinämie mit präferenziell erhöhtem Serum-IgG und Autoantikörper. Zur Remissionsinduktion werden Kortikosteroide eingesetzt, ein schnelles Therapieansprechen unterstützt die Diagnose und führt zu einer guten Langzeitprognose. In der Erhaltungstherapie wird bevorzugt Azathioprin eingesetzt.

Die primär biliäre Zirrhose (PBC) und die primär sklerosierende Cholangitis (PSC) sind immunologische Erkrankungen der Gallenwege. Die PBC hat typischerweise einen gutartigeren Verlauf und ist durch einen langsamen Leberumbau gekennzeichnet. Die PSC führt häufiger und schneller zu einer Leberzirrhose und birgt das Risiko, ein cholangiozelluläres Karzinom zu entwickeln. Beide Erkrankungen werden mit Ursodesoxycholsäure behandelt.

Abstract

Autoimmune hepatitis (AIH) can occur in all age groups. AIH affects women more commonly than men (3:1). Clinical presentation may be an acute hepatitis up to fulminant liver failure, but can also be asymptomatic. AIH is characterized by lymphoplasmacellular infiltrates on liver biopsy, elevated liver enzymes in serum and the absence of active viral markers. Patients characteristically present with hypergammaglobulinemia, elevated serum levels of IgG and autoantibodies. Corticosteroids are the drug of choice for induction of remission, azathioprine the drug of choice for maintenance of remission. Rapid response to immunosuppressive treatment supports the diagnosis and leads to a good long-term prognosis.

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are immune mediated diseases affecting bile ducts. While PBC has a slow progression to cirrhosis and complications mostly will be limited to complications of cirrhosis, PSC additionally carries a high risk of developing cholangiocellular carcinoma. The treatment of choice in PBC and PSC is oral ursodeoxycholic acid which may slow progression of liver disease and may ameliorate lab findings.

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  1. I. Medizinische Klinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 24046, Hamburg, Deutschland

    S. Lüth, C. Weiler-Normann, C. Schramm & A.W. Lohse MD

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  1. S. Lüth
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  2. C. Weiler-Normann
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  3. C. Schramm
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  4. A.W. Lohse MD
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Correspondence to A.W. Lohse MD.

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Lüth, S., Weiler-Normann, C., Schramm, C. et al. Autoimmunerkrankungen der Leber. Internist 50, 310–317 (2009). https://doi.org/10.1007/s00108-008-2254-1

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