Calcific uremic arteriolopathy (calciphylaxis) - Case series and review of clinical features and terminology

We present 3 cases of calciphylaxis, better termed calcific uremic arteriolopathy (CUA), which illustrate typical and atypical presentations of this disorder to discuss its classification, pathophysiology, and treatment based on a review of the literature. Of the 3 patients presenting with biopsy-proven CUA, 1 patient with proximal lesions expired in the setting of upper gastrointestinal bleeding and superinfection of ulcers, a second patient had an extensive distal ulcerative lesion that failed to heal and necessitated lower-extremity amputation, and a third patient with multiple small distal lesions experienced complete resolution of the ulcers. The classification, pathophysiology, and treatment of CUA are detailed here. CUA is a heteroaeneous syndrome which typically follows a progressive course leading to loss of life or limb. Diagnosis requires a high index of suspicion and low threshold for biopsy in at-risk patients. Parathyroidectomy is appropriate to rapidly lower the calcium-phosphorus product in the setting of marked hyperparathyroidism; additional therapy includes the avoidance of purported precipitants, medical lowering of the serum calcium and phosphorus, and promotion of wound healing with hyperbaric oxygen therapy.