Revisiting therapeutic options for calciphylaxis

Calciphylaxis is a disorder of cutaneous microvascular calcification and thrombosis leading to chronic, excruciatingly painful, progressive wounds with a high risk of sepsis and death. The diagnosis and treatment of calciphylaxis presents significant challenges. A poorly understood disease, the management of calciphylaxis has mostly been restricted to wound management and a few novel therapies. Data from patient registries and new studies on causal pathways is stimulating the development of pathogenesis-based medical therapies.

Much needed clinical trials are now underway to examine the safety and efficacy of sodium thiosulfate and other therapeutics for the indication of calciphylaxis. There is emerging data suggesting a potential role of therapeutic anticoagulation in these patients. There has also been a renewed emphasis on patient-oriented outcomes, such as improvement of pain scores and quality-of-life indices.

This review highlights ongoing clinical trials studying therapeutic options in calciphylaxis and emphasizes the causal pathways that led to the development of such therapies.