Calciphylaxis is a severe and complex syndrome involving vascular calcification, thrombosis, and skin necrosis. Although the condition is relatively rare, occurring almost exclusively in patients with advanced (Stage 5) chronic kidney disease (CKD), it can often be fatal. In fact, mortality rates higher than 50 percent are reported within one year of diagnosis, mostly due to septicemia, with an 80 percent mortality rate overall.(1)
Although the term calciphylaxis has been widely used since the 1960s, an alternative and more rigorous term is ‘calcific uremic arteriolopathy (CUA)’, which describes the pathophysiology and predominant pathological findings associated with the condition.(1)
Although calciphylaxis typically occurs in patients with end stage renal disease (ESRD), it can also sometimes be observed in patients with normal renal function. In addition to CKD and ESRD, other known risk factors for calciphylaxis include diabetes mellitus, obesity, hyperphosphatemia, mineral disorders, and exposure to warfarin anticoagulation.(2) The condition is also more prevalent among females and among individuals of Caucasian ethnicity.(3) Overall, the estimated prevalence of calciphylaxis is reported to be up to 4% in patients on dialysis.(2)
Calciphylaxis is a complex disorder with a multifactorial etiology and an ambiguous pathogenesis.(1) Although different treatment strategies are available, their success is often limited. The focus of management, therefore, currently rests in disease prevention among patients with known risk factors. This can be achieved by controlling blood sugars in patients with diabetes mellitus and monitoring calcium-phosphate homeostasis, for example.(1)
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1. Bhambri S, Bhambri A, Del Rosso JA. Calciphylaxis: A review. J Clin Aesthet Dermatol 2008; 1: 38-41
2. Sowers KM, Hayden MR. Calcific uremic arteriolopathy: pathophysiology, reactive oxygen species and therapeutic approaches. Oxid Med Cell Longev. 2010;3(2):109-21.
3. Brandenburg VM, Cozzolino M, Ketteler M. Calciphylaxis: a still unmet challenge. J Nephrol. 2011;24(2):142-8.