Calciphylaxis in 2024

Calciphylaxis is a rare and potentially fatal disease manifested by progressive skin ulcerations due to calcification and obstruction of small-caliber arteries and arterioles. It mainly affects patients with chronic kidney disease treated by dialysis or renal transplantation but also individuals with normal kidney function, in which case it is often associated with chronic inflammatory diseases, neoplasia, primary hyperparathyroidism, and post-bariatric surgery. Necrotized ulcerations can become infected, leading to septic syndrome and a mortality rate of up to 80%. Its incidence varies from one case per 1,000 to one case per 1,500 hemodialysis patients per year, although this is probably underestimated. Clinical manifestations include very painful nodular or plaque-like indurations occurring in the extremities and central to the body. Lesions can also affect the fingers, penis, breasts, and visceral organs such as the lungs, intestines, and eyes. Its management is complex and requires a multimodal, individualized approach, involving close cooperation between nephrologists, dermatologists, surgeons, and other specialists. The aim of this review is to revise old and new aspects of this management while including the control of parameters of mineral and bone metabolism disorders, the replacement of vitamin K antagonists by alternative anticoagulants, the optimization of dialysis prescription, and the use of sodium thiosulfate, as well as new experimental therapies under development. We hope this review will help avoid common pitfalls and provide the highest quality care for patients with calciphylaxis.