Calciphylaxis associated with chronic kidney disease and low bone turnover: management with recombinant human PTH-(1–34)

In 1898 Bryant and White reported features of the condition that was later described as calciphylaxis by Selye in 1961 because of a pathophysiologic resemblance to anaphylaxis [1,2]. Calciphylaxis (or calcific uraemic arteriolopathy) is characterized by medial calcification of small arterioles, intimal proliferation, fibrosis and thrombosis resulting in ischaemia, necrosis and superinfection of the skin and subcutis. When renal function is normal, calciphylaxis has been reported infrequently, associated with conditions such as primary hyperparathyroidism [3]. However, amongst patients with chronic kidney disease (CKD) on dialysis, the annual incidence has been estimated at 1–4%, with an apparent increase over the past decades that may reflect the increased use of calcium-based phosphate binders [4,5]. For patients on dialysis, mortality rates are increased 8-fold if calciphylaxis develops [6]. The mortality of non-ulcerating types is ∼30% and ulcerating types around 80%, and most deaths occur within 6 months [7,8]. In this setting, calciphylaxis is usually associated with poorly controlled secondary hyperparathyroidism. However, cases have been reported in association with low bone turnover and following parathyroid surgery [9,10]. This entity has received little attention and no specific treatment has been suggested.