Calciphylaxis: Clinical presentation, causes and treatment, with case series

Calciphylaxis is a rare condition whereby vascular calcification leads to progressive cutaneous necrosis. It is most commonly observed in the setting of end-stage renal disease, and unfortunately, carries a high morbidity and mortality rate.  Whilst it is thought to be related to calcium homoeostasis, its pathogenesis is poorly understood. Although hypercalcemia, hyperphosphatemia, elevated calcium-phosphate product, secondary hyperparathyroidism, diabetes mellitus and hypercoagulable states have all been suggested as potential causes, it is worth noting that there is a high prevalence of these disorders amongst end-stage renal disease patients, yet calciphylaxis remains an uncommon condition affecting approximately 1-4% of dialysis patients. Little evidence exists for the efficacy of the available treatment options for calciphylaxis, and as such there is no internationally-recognised standard of care. Consequently, the primary focus is currently on prevention through diligent control of serum calcium and phosphate levels in end-stage renal disease patients.
 We present a case series of six patients with end-stage renal disease who developed calciphylaxis, with a discussion of clinical features and management outcomes. In addition, we present a literature review of the breadth of clinical presentations, current hypotheses regarding its pathogenesis, and a review of treatment options.

Dr. Mariya Hamid