Multi-intervention management of calciphylaxis: a report of 7 cases

Abstract

Calcific uremic arteriolopathy (calciphylaxis) is a devastating but rare complication seen predominantly in dialysis patients that often is fatal. Because of the rarity of the disease and the multifactorial nature of its cause, no clinical trials have been conducted to date to determine the best therapy for the condition. We report a case series of 7 patients at a single institution in whom a systematic multi-interventional treatment strategy was implemented, consisting of trigger-agent cessation (calcium-based phosphate binders, alphacalcidol, and warfarin), wound management, and antibiotic therapy, supplemented by intensified hemodialysis (4 hours daily for 7 days followed by 5-6 times weekly), intravenous sodium thiosulfate (12.5-25 g intravenously 3 times a week), and attempted oxygen therapy (given through a face mask or hyperbaric chamber as tolerated by patient circumstance). Treatments selected were based on literature review, consensus discussion, and attempts to address the physiologic disturbances that underlie the condition. All 7 patients identified with biopsy-proven calcific uremic arteriolopathy were treated with this regimen in 2007-2010, with 6 of 7 showing complete recovery. We suggest that consistent implementation of a multi-interventional approach may alter the course of this devastating disease. Further studies are needed to confirm and extend these findings.