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Rückbildung der Retroperitonealfibrose durch eine Kombinationstherapie von Tamoxifen und Steroiden

Regression of retroperitoneal fibrosis to a combined tamoxifen and steroid therapy

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Zusammenfassung

□ Hintergrund

Die idiopathische Retroperitonealfibrose ist gekennzeichnet durch eine fortschreitende Proliferation und Fibrosierung retroperitonealen Gewebes. Durch eine zunehmende Kompression und Ummauerung benachbarter Strukturen kommt es dabei im Verlauf der Erkrankung zu zahlreichen Komplikationen.

□ Fallbeschreibung

Es wird über zwei Patientinnen mit einer idiopathischen Retroperitonealfibrose berichtet. Bei beiden Patientinnen mußte im Verlauf der Erkrankung wegen persistierender Harnabflußstörungen eine Harnleiterlateralisation durchgeführt werden. Zusätzlich entwickelte, sich bei den Patientinnen eine Thrombose der Vena iliaca communis bzw. der Vena cava inferior. Aufgrund des fortschreitenden Charakters der Erkrankung wurde eine Therapie mit Tamoxifen in Kombination mit niedrigdosierten Steroiden eingeleitet. Hierunter kam es bei beiden Patientinnen zu einer Regression der retroperitonealen Veränderungen sowie zu einem Rückgang zuvor bestehender Entzündungszeichen.

□ Schlußfolgerung

Bei Patienten mit einer idiopathischen Retroperitonealfibrose scheint Tamoxifen den Verlauf der Erkrankung günstig zu beeinflussen und eine Regression der bindegewebigen Massen induzieren zu können. Bei den von uns beschriebenen Fällen, insbesondere bei Patienten mit persistierender entzündlicher Aktivität der Erkrankung halten wir eine zusätzliche, niedrigdosierte Steroidmedikation zur Vermeidung einer Bindegewebsneubildung für erforderlich.

Summary

□ Background

Idiopathic retroperitoneal fibrosis is characterized by proliferation and fibrosis of retroperitoneal tissue. It is complicated by obstruction and encasement of retroperitoneal structures.

□ Case Report

We describe two female patients with idiopathic retroperitoneal fibrosis. Both had to undergo lateralization of the ureter because of ureteral obstruction. Also both patients developed thrombosis of the inferior vena cava resp. the common iliac vein. Because of the eventful course of the disease a combined tamoxifen and steroid therapy was started. Hereafter there was a marked regression of the retroperitoneal fibrotic masses and the previous inflammatory signs disappeared.

□ Conclusion

Tamoxifen seems to be effective in the treatment of idiopathic retroperitoneal fibrosis by inducing a regression of the fibrotic masses. Especially in patients with continuous activity of the disease we recommend an additional steroid therapy to prevent a regeneration of the fibrosis.

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Authors and Affiliations

  1. Direktor der Klinik, Innere Medizin IV, Universitätskliniken des Saarlandes, D-66424, Homburg/Saar, Deutschland

    Joachim Allendorff, Werner Riegel & Hans Köhler

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  1. Joachim Allendorff
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  2. Werner Riegel
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  3. Hans Köhler
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Herrn Professor Dr. Hans-Peter Schuster zum sechzigsten Geburtstag

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Allendorff, J., Riegel, W. & Köhler, H. Rückbildung der Retroperitonealfibrose durch eine Kombinationstherapie von Tamoxifen und Steroiden. Med. Klin. 92, 439–443 (1997). https://doi.org/10.1007/BF03042577

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  • DOI: https://doi.org/10.1007/BF03042577

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