Kalziphylaxie

Zusammenfassung

Die Kalziphylaxie ist eine seltene, jedoch mit hoher Morbidität und Mortalität einhergehende Erkrankung, die in erster Linie Dialysepatienten und Patienten mit fortgeschrittener Nierenerkrankung betrifft. Das klinische Bild ist durch äußerst schmerzhafte und häufig nekrotisierende Ulzerationen der Haut charakterisiert, histologisch finden sich ausgeprägte Mediaverkalkungen kutaner Arteriolen, teilweise auch Kalzifikationen im umgebenden Weichteilund Fettgewebe. Eine differenzialdiagnostische Abgrenzung zu kutanen Vaskulitiden, zur Pyoderma gangraenosum oder zu diabetischen Ulzera kann mitunter schwierig sein. In der Vergangenheit bestand die Vermutung, dass die Kalziphylaxie im Rahmen einer renalen High-turnoverOsteopathie bei unkontrolliertem Hyperparathyreoidismus kausal ausgelöst wird. Hier weisen neue Registerdaten jedoch in eine andere Richtung: Eine adyname (Low-turnover-)Osteopathie, Entzündungsprozesse, Hyperphosphatämie, Verkalkungsinhibitormangel und Vitamin-K-Antagonismus scheinen als Risikofaktoren eine dominierendere Rolle zu spielen. Therapeutische Erfolge sind mit Kalzimimetika, Bisphosphonaten und Natrium-Thiosulfat berichtet worden, allerdings nur in Fallberichten bzw. Fallserien - randomisierte Behandlungsstudien zu diesem seltenen Krankheitsbild liegen nicht vor. Potenziell vielversprechend ist die Vitamin-K-Supplementation, essenziell ist eine professionelle Wundbehandlung.

Summary

Calciphylaxis is a rare disease associated with significant morbidity and mortality predominantly affecting patients with advanced chronic kidney disease and especially patients on dialysis. The clinical picture is characterized by severely painful, necrotizing skin ulcerations. In biopsy samples, circumferential media calcifications of small cutaneous arterioles are found, in some cases also calcifications of fat and soft tissues. In the differential diagnosis, vasculitis, pyoderma gangraenosum or diabetic ulcers may have to be considered. In the past, calciphylaxis was thought to be quite strictly related to high bone turnover disease in the context of uncontrolled hyperparathyroidism. New data from registries however point to other directions: Adynamic (low turnover) bone disease, inflammatory processes, hyperphosphatemia, deficiencies in calcification inhibitors and vitamin K antagonism seem to be more prominent risk factors. Therapeutic successes with calcimimetics, bisphosphonates and sodium thiosulfate have been reported, but such observations were limited to case reports or case series. Up to date, no randomized controlled trials have been reported in patients with calciphylaxis. Potentially promising is vitamin K supplementation, adequate and professional wound management is essential.

Schlüsselwörter

Kalziphylaxie - Hyperphosphatämie - Gefäßverkalkungen - Vitamin K - Thiosulfat

Keywords

Calciphylaxis - hyperphosphataemia - vascular calcifications - vitamin K - thiosulfate

• Literatur

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