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Nierenmanifestation bei systemischer Sklerose

Renal manifestation in systemic sclerosis

  • Leitthema
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Der Nephrologe Aims and scope

Zusammenfassung

Die renale skleroderme Krise (SRK) stellt die gefürchtetste Nierenmanifestation bei Patienten mit systemischer Sklerose dar und verläuft unter dem klinischen Bild einer thrombotischen Mikroangiopathie. Sie geht mit reduziertem Nieren- und Gesamtüberleben einher. Risikofaktoren für die SRK bestehen in ausgedehntem, rasch progredientem Hautbefall, Gelenkkontrakturen, positivem Anti-RNA-Polymerase-III-Antikörper-Nachweis, vorangegangener hoch dosierter Glukokortikoidmedikation sowie dem Vorliegen von Umständen, die den renalen Blutfluss vermindern. Durch eine schnelle Diagnosestellung, den Einsatz von ACE-Hemmern und supportive Nierenersatztherapie hat die sich die Prognose signifikant verbessert. Trotzdem haben Patienten nach SRK ein schlechteres Überleben als Patienten mit systemischer Sklerose ohne SRK, auch unter allen Formen der Nierenersatztherapie im Vergleich zu Patienten mit anderen Ursachen für eine terminale Niereninsuffizienz. Viele neue therapeutisch nutzbare Targets wurden jüngst identifiziert und müssen auf ihre Wertigkeit hinsichtlich der Behandlung der SRK untersucht werden.

Abstract

Scleroderma renal crisis (SRC) remains the most feared renal complication in patients with systemic sclerosis, presenting clinically as thrombotic microangiopathy with reduced kidney and patient survival. Rapidly progressive dermal fibrosis, joint contractures, positive detection of anti-RNA polymerase III autoantibodies, antecedent high-dose glucocorticoid medication as well as reduced renal blood flow were identified as risk factors for SRC. Prompt recognition and initiation of treatment with angiotensin-converting enzyme (ACE) inhibitors and supportive renal replacement therapy provides the best chance for a favorable outcome. Nevertheless, survival after SRC is worse than in patients with systemic sclerosis without SRC and also poorer with all forms of renal replacement compared to patients with other causes of end-stage renal disease. A variety of novel therapeutic targets were recently discovered and remain to be evaluated concerning their benefits for treatment of SRC.

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Authors and Affiliations

  1. Medizinische Klinik III (Nephrologie, Hypertensiologie, Rheumatologie), Sana Klinikum Offenbach GmbH, KfH Nierenzentrum Offenbach, Starkenburgring 66–70, 63069, Offenbach, Deutschland

    K. de Groot

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K. de Groot gibt an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine vom Autor durchgeführten Studien an Menschen oder Tieren.

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M. Haubitz, Fulda

U. Heemann, München

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de Groot, K. Nierenmanifestation bei systemischer Sklerose. Nephrologe 11, 198–204 (2016). https://doi.org/10.1007/s11560-016-0050-9

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