Multidisciplinary management of calciphylaxis: a series of 5 patients at a single facility

CEN Case Rep. 2020 May;9(2):122-128. doi: 10.1007/s13730-019-00439-8. Epub 2019 Dec 17.

Abstract

Calciphylaxis is a rare and severe disease that manifests with painful skin ulceration and necrosis. Herein, we report five patients of hemodialysis patients with skin biopsy-proven calciphylaxis at a single facility. One patient had undergone parathyroidectomy (PTx) due to severe secondary hyperparathyroidism, four had been treated with vitamin D receptor activators, and two were on warfarin therapy. All patients had hyperphosphatemia, and one had hypercalcemia. The intact parathyroid hormone level at diagnosis was 2 pg/ml in the patient after PTx, while three patients were within the target range. The average period after diagnosis of calciphylaxis was 2 months. Skin lesions were present on the thighs and lower legs in two patients, and on the dorsum of the foot in one patient. In skin biopsy, calcification was found in the arteriolar media in four patients, and calcium (Ca) was deposited in the dermal lesion in one patient. All patients received local cures, surgical debridement, antibiotics to control infectious diseases, and strict control of serum Ca and phosphate. Calcimimetics were used in all patients except one who had undergone PTx one month before, sodium thiosulfate was used in 4 patients, and low Ca dialysate was used in three patients. The average follow-up period was 7.4 months. Four patients were cured, and one died due to infection. We suggest that multidisciplinary management for infectious diseases, surgical debridement, strict control of mineral and bone markers from the early stage, and elimination of risk factors may improve the course of calciphylaxis, which is a life-threatening disease.

Keywords: Calcific uremic arteriolopathy; Calcimimetics; Calciphylaxis; Hyperphosphatemia; Surgical debridement.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Calciphylaxis / drug therapy*
  • Calciphylaxis / etiology
  • Calciphylaxis / pathology
  • Calciphylaxis / surgery*
  • Combined Modality Therapy
  • Female
  • Humans
  • Hypercalcemia / diagnosis
  • Hyperparathyroidism, Secondary / complications
  • Hyperparathyroidism, Secondary / drug therapy
  • Hyperparathyroidism, Secondary / surgery*
  • Hyperphosphatemia / diagnosis
  • Interdisciplinary Communication
  • Male
  • Middle Aged
  • Necrosis / etiology
  • Necrosis / pathology
  • Parathyroidectomy / adverse effects
  • Receptors, Calcitriol / agonists*
  • Renal Dialysis / adverse effects
  • Skin Ulcer / etiology
  • Skin Ulcer / pathology
  • Treatment Outcome
  • Warfarin / therapeutic use

Substances

  • Receptors, Calcitriol
  • Warfarin