Issues

Calciphylaxis

Journal: Osteologie
ISSN: 1019-1291
Topic:

Kalzium-Phosphat-Stoffwechsel und Knochen

Issue: Issues of 2017 (Vol. 26): HeIssue 3 2017 (131-191)
Pages: 147-152

Calciphylaxis

M. Ketteler (1), A. Raabe (1), S. Piltz (2)

(1) III. Medizinische Klinik: Nephrologie und Dialyse, Klinikum Coburg GmbH, Coburg; (2) Klinik für Unfallchirurgie und Orthopädie, Klinikum Coburg GmbH, Coburg

Keywords

vitamin K, hyperphosphataemia, calciphylaxis, vascular calcifications, thiosulfate

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.

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