Rapid improvement of calciphylaxis after intravenous pamidronate therapy in a patient with chronic renal failure

Calciphylaxis, also called calcific uraemic arteriolopathy, is a rare disease characterized by medial calcification of the small arteries and ischaemia of the subcutaneous tissue, often leading to necrosis of subcutaneous fat and skin. It affects mainly women with chronic renal insufficiency and/or obesity. According to recent studies, calciphylaxis seems to occur more frequently than previously believed, with an incidence of 1% per year [1] and a prevalence of 4% in dialysis patients [2]. The pathogenesis of calciphylaxis is poorly understood and its treatment is largely empirical and somewhat controversial. Recent studies have emphasized the crucial role of a multidisciplinary therapeutic approach focusing on the correction of the underlying abnormalities of the calcium and phosphorus plasma concentrations (using non-calcium-containing phosphate binders), local wound care with debridement of necrotic tissues and aggressive treatment of infectious complications [3]. The utility of parathyroidectomy, corticoid therapy and hyperbaric oxygen therapy remains controversial. However, despite intensive combined treatments, the prognosis of calciphylaxis remains poor: the overall 1 year survival is 45% and the 5 year survival is 35%, with a relative risk of death of 8.5 compared with other dialysis patients [4]. Bisphosphonates have a powerful inhibitory effect on osteoclast activity and bone resorption and are largely used in the treatment of osteoporosis, tumoral hypercalcaemia and Paget’s disease. Some previous studies have shown that bisphosphonates also have beneficial effects on the evolution of experimental calciphylaxis [5,6] and tumoral calcinosis with systemic inflammatory response [7]. According to these latter observations, we decided to treat a woman with chronic renal failure and rapidly worsening calciphylaxis with pamidronate. This treatment brought a spectacular and rapid improvement of the patient’s clinical condition and a rapid decrease of the inflammatory syndrome. We report this case, which is, to our knowledge, the first one in which a bisphosphonate has been used to treat a patient with calciphylaxis.