Management of patients with calciphylaxis: current perspectives

Abstract

Calciphylaxis (CP) is a rare disorder presenting with painful ischemic skin ulcerations. Its association with end-stage renal disease, but also non-uremic cases have been described. Several risk factors, like metabolic syndrome, calcium-phosphate imbalance, anticoagulative medication and female gender have been discussed. Multidisciplinary therapeutic approaches are necessary since evidence-based guidelines are missing due to lack of pathophysiological understanding of the disease and the absence of studies with large patient cohorts. However, strategies for local wound therapy, reduction of risk factors and systemic therapies are being developed. Changes in duration, frequency and method of hemodialysis and specific medication to lower the calcium upload can be effective in uremic CP cases. Systemic treatments with cinacalcet, sodium thiosulfate or bisphosphonates have been applied successfully in CP patients, but large placebo-controlled randomized trials are still needed to gather better insight into this fatal disease.