Review: update on the management of calciphylaxis.

ABSTRACT

Calciphylaxis is a disease of significant morbidity and mortality, predominantly affecting dialysis patients. The term 'calciphylaxis' was coined by Seyle et al. in 1961 to describe calcium deposition in the skin and subcutaneous soft tissue of uremic rats in response to 'triggers' (e.g. trauma, metallic salts) after exposure to 'sensitizing agents' (e.g. vitamin D and parathyroid hormone). In humans, calciphylaxis, however, is not a disorder of induced hypersensitivity. Instead, it is a disorder of cutaneous microvascular occlusion caused by thrombosis and calcification. Progressive, excruciatingly painful, non-healing wounds develop in these patients, pre-disposing them to high risk of sepsis and death. Calciphylaxis has no approved therapies. Increased awareness and research in this field have facilitated identification of risk factors and causation pathways. Development of therapeutic options and wound care management, however, are still at a nascent stage. Certain therapies have shown a promise that needs evaluation in prospective clinical trials. It is hoped that ongoing research will help us better understand the pathogenesis of this complex disease and develop efficacious treatment options. In this review, we outline the components involved in calciphylaxis diagnosis and treatment.