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Nephrogene systemische Fibrose

Nephrogenic Systemic Fibrosis

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Zusammenfassung

Die nephrogene systemische Fibrose ist eine außerordentlich invalidisierende und emotional belastende Erkrankung. Sie wurde bisher ausschließlich bei Patienten mit einer akuten oder chronischen Niereninsuffizienz beobachtet. Daneben war fast allen Patienten zuvor gadoliniumhaltiges Kontrastmittel appliziert worden. Zu den frühen Symptomen gehören Schmerzen, Jucken und Schwellung vorwiegend der Extremitäten. Im weiteren Verlauf kommt es zu einer progredienten Fibrosierung der Haut und anderer Organe. Die Diagnose wird in Zusammenschau von Anamnese, Klinik und histologischen Veränderungen der Haut gestellt. Hinsichtlich der Prävention ist die Vermeidung gadoliniumhaltiger Kontrastmittel bei hochgradiger Niereninsuffizienz entscheidend. Bisher ist keine wirksame Therapie der Erkrankung bekannt. Mögliche Therapieansätze, die in Einzelfällen erfolgreich waren, beinhalten u.a. eine schnelle Wiederherstellung der Nierenfunktion, Physiotherapie und die extrakorporale Photopherese.

Abstract

Nephrogenic systemic fibrosis is a highly debilitating and emotionally depressing disorder. So far, it has exclusively occurred in patients suffering from acute or chronic renal failure. Most patients had been exposed to gadolinium- based contrast agents. Early symptoms include pain, swelling and pruritus, predominantly of the extremities. Fibrosis of the skin and other organs develops in the further course of the disease. The diagnosis is made based on the synopsis of medical history, clinical and dermatohistological findings. To avoid the application of gadolinium-based contrast agents in patients with highly impaired renal function is the best measure to prevent the disease. An effective therapy has not yet been established. Possible therapies, which have been successful in individual cases, comprise, among others, a quick restoration of renal function, physiotherapy, and extracorporeal photopheresis.

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Authors and Affiliations

  1. Klinik für Nephrologie, Zentrum für Innere Medizin, Universitätsklinikum Essen, Universität Duisburg-Essen, Essen, Germany

    Andreas Kribben

  2. Klinik für Nephrologie, Zentrum für Innere Medizin, Universitätsklinikum Essen, Universität Duisburg-Essen, Hufelandstraße 55, 45122, Essen, Germany

    Andreas Kribben

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  1. Stefan Becker
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  2. Oliver Witzke
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  3. Andreas Kribben
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Correspondence to Andreas Kribben.

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Becker, S., Witzke, O. & Kribben, A. Nephrogene systemische Fibrose. Med Klin 104, 204–209 (2009). https://doi.org/10.1007/s00063-009-1033-7

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  • DOI: https://doi.org/10.1007/s00063-009-1033-7

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