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Nephrogene systemische Fibrose

Nephrogenic systemic fibrosis

  • Leitthema: Kontrastmittelsicherheit
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Zusammenfassung

Ein neues, Skleromyxödem-ähnliches Krankheitsbild wurde erstmals 1997 beobachtet und im Jahre 2000 publiziert. Es trat nur bei Patienten mit eingeschränkter Nierenfunktion (glomeruläre Filtrationsrate [GFR] <30 ml/min/1,73 m2) auf, weshalb der Terminus nephrogene fibrosierende Sklerose gewählt wurde. Erst 2006 konnte ein Zusammenhang dieses Krankheitsbildes mit einer vorausgegangenen kontrastverstärkten Magnetresonanztomographie- (MRT-)Untersuchung in Zusammenhang gebracht werden. Da die Erkrankung nicht auf die Haut und Muskulatur begrenzt war, sondern auch innere Organe (u. a. Lunge, Herz, Augen) einbezog, wurde nun der Begriff nephrogene systemische Fibrose (NSF) eingeführt. Bisher sind ca. 215 Patienten mit dieser Hauterkrankung an internationale Register gemeldet worden. Im Zentrum der Erkrankung steht eine Verhärtung der Haut, die an den distalen Extremitäten beginnt und nach proximal fortschreitet. Einbezogene Gelenke können in Beugekontrakturstellung fixiert werden und zu erheblichen Behinderungen mit Rollstuhlpflichtigkeit oder Bettlägerigkeit führen. Die Haut zeigt eine typische Histologie. Hinsichtlich der Pathogenese sind nur Teilaspekte bekannt, wobei derzeit dem gadoliniumhaltigen Kontrastmittel eine Schlüsselrolle zugesprochen wird. Aus der Vielzahl der eingesetzten Therapien hat sich bisher keine Erfolg versprechende Behandlungsoption herauskristallisiert. Die Prognose ist mit einer Sterblichkeit von bis zu 28% schlecht, während spontane Remissionen selten sind. Daher kommt der Prophylaxe eine große Rolle zu: Patienten mit einer Niereninsuffizienz im Stadium 4 (GFR <30 ml/min/1,73 m2) sollten nur dann nach entsprechender Aufklärung einer kontrastverstärkten MR-Untersuchung unterzogen werden, wenn keine alternativen bildgebenden Verfahren zur Verfügung stehen.

Abstract

A scleromyxedema-like disease was recognized in 1997. In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance imaging (MRI). All patients had acute or chronic severe renal insufficiency with a glomerular filtration rate (GFR) <30 ml/min per 1.73 m2. So far a total of about 215 patients with this new skin disorder have been reported to international registries. The skin thickening has a typical histology and begins in the peripheral extremities and progresses proximally, including also the abdominal wall and the head in some patients. NSF involves not only the skin, but also the muscles and other organs (e.g., lungs, heart, eyes) in some patients. Therefore the term nephrogenic systemic fibrosis (NSF) was introduced. Skin fibrosis and sclerosis are usually progressive with disabling contractures of involved joints (knees, hands, feet). NSF may be lethal in up to 28% of patients. Spontaneous remissions are rare. No generally accepted treatment is available. So far, the pathogenesis is not well understood. One hypothesis supposes a role of gadolinium liberated from the contrast agents. As patients with acute or chronic advanced renal failure (GFR <30 ml/min per 1.73 m2) including those with hepatorenal dysfunctions are at high risk to develop NSF after exposure to gadolinium-based contrast agents, contrast-enhanced MRI should be avoided in this group and alternative diagnostic procedures should be used whenever possible.

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  1. Schwerpunkt Nephrologie,Medizinische Klinik und Poliklinik I, Klinikum Großhadern der Ludwig-Maximilians-Universität München, Marchioninistraße 15, 81377, München, Deutschland

    W. Samtleben

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Samtleben, W. Nephrogene systemische Fibrose. Radiologe 47, 778–784 (2007). https://doi.org/10.1007/s00117-007-1545-1

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