Retroperitoneale Fibrose

 

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Zusammenfassung

Die retroperitoneale Fibrose (RPF) ist eine entzündlich-fibrosierende Erkrankung des hinteren Bauchraumes. Mit einer Prävalenz von 1 - 2/200 000 gehört sie zu den seltenen Erkrankungen. Leitlinien zur Diagnostik und Therapie gibt es bis dato nicht. Unbehandelt kann die Erkrankung zum Tode führen. Seit Anfang 2006 koordiniert die Klinik für Urologie im HELIOS Klinikum Wuppertal den Aufbau eines bundesweiten Patientenregisters als Vorbereitung für prospektive Therapiestudien und die Erstellung von Diagnoseempfehlungen. Die Ätiologie der RPF ist weitgehend ungeklärt. RPF-Patienten weisen teilweise Erkrankungen aus dem autoimmunen Formenkreis auf. Ein autoimmunes Geschehen als Krankheitsursache wird vermutet. Der Nachweis von Autoantikörpern und die histologische Ähnlichkeit zu Vaskulitiden unterstützen diese These. Neben Allgemeinsymptomen zeigen die Patienten im Krankheitsverlauf lokalisierte Symptome (Flankenschmerzen, Beinödeme, abdominelle Beschwerden), bedingt durch die verdrängende Wirkung der fibrotischen Plaques. Laborchemisch fallen eine erhöhte Blutsenkungsgeschwindigkeit, erhöhtes C-reaktives Protein und ggf. eine moderate Anämie auf. Differenzialdiagnostisch sollten Malignome histologisch ausgeschlossen werden. In der Bildgebung (i. v.-Urogramm, CT, MRT) findet man eine retroperitoneale Raumforderung, die die großen Gefäße sowie die Harnleiter ganz oder teilweise ummauert, komprimiert und verdrängt. Therapeutisch muss die Funktion der betroffenen Hohlorgane durch Stents bzw. Harnleiterschienen wiederhergestellt werden, gefolgt von einer immunsuppressiven Therapie. Führt diese nicht zu einem dauerhaften Erfolg, stehen operative Verfahren zum Schutz der Harnleiter vor Kompression zur Verfügung. Ggf. wird ein Harnleiterersatz oder eine Autotransplantation der Niere erforderlich. Aufgrund des chronisch rezidivierenden Verlaufs ist oftmals eine lebenslange Nachsorge angezeigt. Einer fachübergreifenden und überregionalen Kooperation kommt bei der weiteren Erforschung der Erkrankung hohe Bedeutung zu.

Abstract

Retroperitoneal fibrosis (RPF) is characterised by inflammatory fibrotic processes affecting the retroperitoneal structures. Its prevalence of 1 - 2/200 000 makes it a rare disease. To date, there are no guidelines for the diagnosis of or therapy for the disease. If untreated, the disease may be fatal. In 2006, the Department of Urology of the HELIOS Klinikum Wuppertal undertook to establish a nationwide patient registry, which would facilitate prospective therapy trials and the drafting of recommendations for diagnostic procedures. The pathogenesis of the disease is still unclear. Since some RPF-patients present with associated autoimmune diseases, autoimmune processes are suspected to play a role in causing the disease. The presence of autoantibodies and histological similarities with vasculitis support this hypothesis. Following initial general symptoms, patients display localised symptoms (flank pain, leg oedema, abdominal discomfort), caused by the displacing effect of the fibrotic plaques. Laboratory tests show elevated ESR and C-reactive protein and in some cases a moderate anaemia. Histological examinations should be undertaken to rule out the presence of malignant tumours. Radiological diagnostics (excretory urography, CT, MRI) show a retroperitoneal mass which blocks, compresses and displaces, completely or in part, the large vessels and the ureter. Initial therapy aims at restoring the function of the affected hollow organs through the application of (ureteric) stents, followed by immunosuppressive therapy. If drug therapy is unsuccessful, surgical procedures will follow to protect the ureter from compression. In some cases, ureteral replacement or an autotransplant of the kidney may be necessary. Life-long observation of the patients is necessary, as the disease may be chronic and relapsing. Interdisciplinary and nationwide cooperation is of crucial importance to further investigate this disease.

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