Calciphylaxis: risk factors, diagnosis, and treatment

Abstract

Calciphylaxis is a rare but devastating condition that has continued to challenge the medical community since its early descriptions in the scientific literature many decades ago. It is predominantly seen in patients with chronic kidney failure treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal kidney function. In this review, we discuss the available medical literature regarding risk factors, diagnosis, and treatment of both uremic and nonuremic calciphylaxis. High-quality evidence for the evaluation and management of calciphylaxis is lacking at this time due to its rare incidence and poorly understood pathogenesis and the relative paucity of collaborative research efforts. We hereby provide a summary of recommendations developed by a multidisciplinary team for patients with calciphylaxis.

Keywords: Calcific uremic arteriolopathy; calciphylaxis; review; risk factors; sodium thiosulfate; warfarin.

Figure 1. Morphology of calciphylaxis lesions

Figure 2. Histopathology of calciphylaxis

Course basophilic medial calcification of small arteries as demonstrated by Hematoxylin & Eosin stain (400×) and highlighted by von Kossa histochemical stain (200×) (Panel A-B). Septal panniculitis and subcutaneous fat necrosis with presence of subtle finely granular basophilic calcium deposits (400×, Hematoxylin & Eosin, Panel C). A von Kossa histochemical stain aids in the detection of interstitial calcium deposits, which may not be identified on routine histologic sections (200×, Panel D).

Figure 3. Professor Selye's experimental calciphylaxis model