Multifaceted approach to a rare clinical case of calciphylaxis in a renal transplant recipient

G Ital Nefrol. 2019 Sep 24;36(5):2019-vol5.

Abstract

Calcific uremic arteriolopathy (CUA) is a highly morbid condition usually found in ESRD patients that has rarely been reported after renal transplantation and renal function restoration. Furthermore, little is known about the optimal management of CUA in this setting. Herein, we report on the clinical case of AB, a 70-year-old woman who developed CUA after renal transplantation and renal function restoration. However, other risk factors for CUA such as diabetes and warfarin treatment, due to mechanical aortic valve implantation, were present. Thirty-eight months after renal transplantation she developed erythema and livedo reticularis in both legs and a gradually enlarging skin ulcer in the right leg. A skin biopsy of the ulcer showed features compatible with the CUA, such as sub-intimal calcification and luminal obstruction of the small dermal arterioles, tissue ischemia and signs of adipocytes degeneration. A multidisciplinary approach was adopted, including medical and non-medical treatments such as surgical debridement and vacuum-assisted closure therapy. Medical treatments included a five weeks course of once a week intravenous infusion of pamidronate and intravenous sodium thiosulfate (STS) at increasing doses. Four months after beginning the therapy with STS, a complete healing of the ulcer on the right leg and the disappearance of the livedo reticularis on the left leg was noted. In conclusion, although rare CUA may develop also in renal transplanted patients, a timely and combined therapeutic approach is essential for its resolutive treatment. Sodium thiosulfate therapy has proven to be effective and tolerated.

Keywords: calcific uremic arteriolopathy; calciphylaxis; renal transplantation; sodium thiosulfate.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Anticoagulants / therapeutic use
  • Bone Density Conservation Agents / administration & dosage
  • Calciphylaxis / etiology
  • Calciphylaxis / therapy*
  • Chelating Agents / administration & dosage
  • Combined Modality Therapy / methods
  • Diabetes Mellitus
  • Female
  • Humans
  • Kidney Transplantation / adverse effects*
  • Leg Ulcer / etiology
  • Leg Ulcer / therapy*
  • Livedo Reticularis / etiology
  • Livedo Reticularis / therapy
  • Pamidronate / administration & dosage
  • Rare Diseases / etiology
  • Rare Diseases / therapy*
  • Risk Factors
  • Thiosulfates / administration & dosage
  • Warfarin / therapeutic use

Substances

  • Anticoagulants
  • Bone Density Conservation Agents
  • Chelating Agents
  • Thiosulfates
  • Warfarin
  • sodium thiosulfate
  • Pamidronate