Zusammenfassung
Die autoimmune Hepatitis (AIH), die primär biliäre Zirrhose (PBC) und die primär sklerosierende Cholangitis (PSC) sowie deren Overlapsyndrome werden als autoimmune Lebererkrankungen angesehen. Diese Erkrankungen stellen eine wichtige Differenzialdiagnose akuter und chronischer Hepatopathien dar. Die Diagnose autoimmuner Lebererkrankungen und deren Differenzierung kann durch ein diagnostisches Stufenschema mit sequenzieller Bestimmung der relevanten Autoantikörper, dem Ausschluss anderer Lebererkrankungen und schließlich bildgebender Diagnostik und Histologiegewinnung systematisch erleichtert werden. Für die AIH gilt, dass eine immunsuppressive Therapie bei rechtzeitigem Therapiebeginn die Entwicklung einer Leberzirrhose verhindert und - selbst bei Vorliegen einer Zirrhose - die Prognose entscheidend verbessert. Für die anderen autoimmunen Lebererkrankungen ist der Nutzen einer immunsuppressiven Therapie bisher nicht sicher nachgewiesen, so dass hier individuelle Therapieentscheidungen notwendig sind. Ursodesoxycholsäure (UDCA) scheint aber vor allem in frühen Stadien der PBC den Krankheitsprozess zu verlangsamen.
Abstract
Autoimmune Hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and overlap syndromes of these three disease enteties are regarded as autoimmune liver diseases. These conditions are important differential diagnoses of elevated liver function tests as about 10 % of liver transplantations in Europe and North America are for these indications. The diagnosis is often difficult but can be facilitated by sequential measurement of relevant autoantibodies, exclusion of other liver disease, ultrasound, ERCP and liver histology. In AIH immunosuppressive therapy has been shown to prevent or stop the development of cirrhosis and improve the prognosis of the patients decisively. In other autoimmune liver diseases this evidence is missing making individual therapeutic descisions necessary. Ursodesoxycholic acid (UDCA) seems to slow disease progression in particular in early stages of PBC.
Schlüsselwörter
Autoimmune Hepatitis - primär biliäre Zirrhose - primär sklerosierende Cholangitis - Overlapsyndrome - immunsuppressive Therapie - Lebertransplantation
Key words
Autoimmune hepatits - primary biliary cirrhosis - primary sclerosing cholangitis - overlap syndromes - immunosuppressive therapy - liver transplantation
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Prof. Dr. Ansgar W. Lohse
I. Medizinische Klinik und Poliklinik, Johannes-Gutenberg-Universität Mainz
Langenbeckstr. 1
55131 Mainz
Email: lohse@mail.uni-mainz.de
