Kalziphylaxie

Zusammenfassung

Die Kalziphylaxie (kalzifizierende urämische Arteriolopathie, Englisch: calcific uremic arteriolopathy, CUA) ist eine seltene Erkrankung an der Schnittstelle zwischen Nephrologie, Dermatologie und Angiologie. Die CUA tritt meist bei erwachsenen chronischen Dialysepatienten auf, anekdotisch kommen auch Fälle bei Patienten ohne relevante Nierenerkrankung vor. Klinisch ist die CUA charakterisiert durch eine klassische kaskadenartige Entwicklung, die mit stark schmerzhaften oberflächlichen Missempfindungen beginnt und sich über Livedo-artige Hautveränderungen begleitet von einer derben Induration zu offenen Ulzerationen weiterentwickeln kann. Der histopathologische Befund ist von einer Pannikulitis mit oft zirkumferenten Kalzifikationen innerhalb der Wand von kutanen Arteriolen gekennzeichnet, was gemeinsam mit Endothelabschilferungen zu Gefäßokklusionen und Nekrosen führen kann. Die Prognose der CUA ist von einer hohen Morbidität und Mortalität gekennzeichnet. Die Ätiologie der Erkrankung ist nur teilweise aufgeklärt. Auffallend ist, dass ein hoher Anteil an betroffenen Patienten unter einer Therapie mit Vitamin-K-Antagonisten zur oralen Dauerantikoagulation stand. In der Therapie fehlen bisher evidenzbasierte Standards. Konsequente Schmerztherapie und ein gutes Wundmanagement sind Eckpfeiler der Therapie. Registerstudien wie das deutsche Kalziphylaxieregister (www.calciphylaxis.net) können helfen, Risikofaktoren aufzudecken, Expertenwissen auszutauschen, lokale Behandlungszentren zu identifizieren und bis zur Etablierung von studienbasierter Therapieevidenz zumindest eine „good clinical practice“ zu definieren.

Abstract

Calciphylaxis (calcific uremic arteriolopathy, CUA) is a rare disease at the interface of nephrology, dermatology and cardiovascular medicine. CUA typically occurs in chronic dialysis patients. However, anecdotal reports also exist about cases in patients without relevant kidney disease. Clinically CUA is characterized by the stepwise development of superficial painful sensations and cutaneous lesions similar to livedo reticularis. Skin necrosis and ulceration represent the full-blown, “late” clinical picture. Panniculitis and circumferential calcification of cutaneous arterioles dominate the histological picture together with endothelial detachment. The prognosis of CUA is poor due to high morbidity and mortality largely resulting from underlying cardiovascular disease or septicemia. The aetiology of CUA is incompletely understood. Previous oral anticoagulation with vitamin K antagonists is considered as a risk factor. Unfortunately, evidence-based therapeutic options are absent, since controlled treatment trials have not been conducted yet. Long-term pain and wound management are mandatory. In the absence of controlled prospective trials registry studies such as the German CUA registry (www.calciphylaxis.net) support data collecting and analysis upon good clinical practice and may stimulate exchange of expertise and networking.

Schlüsselwörter

Kalziphylaxie - kalzifizierende urämische Arteriolopathie - Kalzifikation - Dialyse - terminales Nierenversagen

Keywords

calciphylaxis - calcific uremic arteriolopathy - calcification - dialysis - end-stage renal disease

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