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Lebertransplantation bei primär sklerosierender Cholangitis und Colitis ulcerosa

Eine therapeutische Herausforderung

Liver transplantation in patients with PSC and ulcerative colitis

A clinical challenge

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Zusammenfassung

Das langfristige Überleben ist sowohl bei asymptomatischen wie auch bei symptomatischen Patienten mit primär sklerosierender Cholangitis (PSC) beeinträchtigt. Dies resultiert unter anderem aus der erhöhten Inzidenz von cholangiozellulären Karzinomen auf dem Boden der Gallenwegsveränderungen. Die rechtzeitige Indikationsstellung zur Lebertransplantion ist eine klinische Herausforderung. Zum einen ist der Verlauf der PSC oft sehr langsam progredient und wird dann akut durch das Auftreten von septischen Cholangitiden beschleunigt. Dies wird in vielen Fällen nicht ausreichend gut durch die bestehenden Prognosescores abgebildet und gilt auch für den MELD-Score („Model for End-Stage Liver Disease“), der die Grundlage für die Allokation von Spenderorganen bei Lebertransplantationskandidaten ist. Aus diesem Grund ist für Transplantationskandidaten mit einer PSC eine Ausnahmeregelung eingeführt worden („Exceptional MELD“). Zum anderen rechtfertigt allein das erhöhte Risiko einer Karzinomentwicklung nicht die prophylaktische Indikation zur Lebertransplantation. Die Ergebnisse nach Lebertransplantation bei PSC sind im Trend besser als für andere Indikationen, obwohl bis zu 40% der Patienten nach Lebertransplantation eine erneute PSC oder ein PSC-ähnliches Syndrom entwickeln. Bei PSC-Patienten, bei denen sich ein Gallenwegskarzinom entwickelt hat, soll eine Lebertransplantation nur innerhalb klinischer Studien durchgeführt werden. Die Prävalenz chronisch-entzündlicher Darmerkrankungen nach Lebertransplantation bei Patienten mit PSC wird mit mehr als 65% (bis 100%) angegeben und zumeist als Colitis ulcerosa klassifiziert. Die Proktokolektomie kann auch nach Transplantation sicher durchgeführt werden. Inwieweit die Patienten nach Proktokolektomie auch einen verbesserten Verlauf der rekurrenten PSC zeigen, ist derzeit nicht geklärt.

Abstract

The survival of patients with primary sclerosing cholangitis (PSC) is reduced in part because of an increased incidence of cholangiocellular carcinoma. However, the timing of liver transplantation represents a considerable clinical challenge. The clinical course of PSC is frequently slowly progressive and can develop acutely with the occurrence of septic cholangitis. In most cases this situation is not adequately reflected by established prognostic models, including the Model for End-Stage Liver Disease (MELD) score, which is the basis for donor organ allocation in liver transplantation candidates. Therefore, an exceptional-case status has been defined for certain transplantation candidates with PSC („Exceptional MELD“). The increased risk of cholangiocarcinoma alone does not warrant prophylactic liver transplantation. The overall results after liver transplantation for PSC are generally better than for other indications, although up to 40% develop recurrent PSC or a PSC-like syndrome. In PSC patients with cholangiocarcinoma, liver transplantation should not be performed outside of clinical studies. The prevalence of chronic inflammatory bowel disease after liver transplantation in patients with PSC is reported to exceed 65% (up to 100%) and is usually classified as ulcerative colitis. Proctocolectomy can be safely performed after transplantation. However, the extent to which patients have an improved course of recurrent PSC after proctocolectomy has yet to be established.

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Authors and Affiliations

  1. Campus Virchow-Klinikum, Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Charité – Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland

    O. Guckelberger, U.P. Neumann & P. Neuhaus

  2. Klinik für Gastroenterologie, Hepatologie und Endokrinologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    M. Manns & C.P. Strassburg

Authors
  1. O. Guckelberger
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  2. U.P. Neumann
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  3. P. Neuhaus
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  4. M. Manns
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  5. C.P. Strassburg
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Correspondence to O. Guckelberger.

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Guckelberger, O., Neumann, U., Neuhaus, P. et al. Lebertransplantation bei primär sklerosierender Cholangitis und Colitis ulcerosa. Gastroenterologe 4, 419–426 (2009). https://doi.org/10.1007/s11377-009-0293-3

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  • DOI: https://doi.org/10.1007/s11377-009-0293-3

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