Calciphylaxis

CALCIPHYLAXIS IS A RARE, LIFE-THREATENING SYNDROME OF VASCULAR calcification characterized by occlusion of microvessels in the subcutaneous adipose tissue and dermis that results in intensely painful, ischemic skin lesions. Once calciphylaxis has been diagnosed, the prognosis is generally poor (survival, <1 year).1-3 The disorder, which is underrecognized,4 typically affects patients with end-stage renal disease (ESRD),2,5 a population with a high prevalence of extraskeletal calcifications. A clear majority of such calcifications do not represent calciphylaxis, which cannot be placed on a simple continuum of vascular calcification. However, an improved understanding of vascular calcification has helped to elucidate the pathogenesis of calciphylaxis and promising approaches to treatment. Calciphylaxis also occurs in patients with earlier stages of chronic kidney disease,2,5 acute kidney injury,6 or prior receipt of a kidney transplant,7 and in rare cases, it occurs in patients with normal kidney function.2,5,8,9 We therefore prefer the name calciphylaxis to calcific uremic arteriolopathy, which is another name for this entity.10 This review presents the current understanding of calciphylaxis and provides a framework for its interdisciplinary management. Copyright © 2018 Massachusetts Medical Society.