Abstract

Calcific uremic arteriolopathy (CUA) is a rare and potentially fatal disorder of calcification involving subcutaneous small vessels and fat in patients with renal insufficiency. We describe the successful use of intravenous sodium thiosulfate (STS) for the treatment of CUA in two patients. The first case was complicated by the development of a severe anion gap metabolic acidosis, which was accompanied by a seizure. Both patients had complete wound healing within five months. Although STS should be considered in the treatment of CUA, little is known about pharmacokinetics and additional studies are required to determine dosing strategies to minimize severe potential side effects.

Highlights

  • Calcific uremic arteriolopathy (CUA), known as calciphylaxis, is a rare but potentially fatal disorder associated with renal insufficiency

  • We describe the successful use of intravenous sodium thiosulfate (STS) for the treatment of CUA in two patients

  • We reported on 2 cases of CUA with anion gap metabolic acidosis (AGMA) from IV STS

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Summary

Introduction

Calcific uremic arteriolopathy (CUA), known as calciphylaxis, is a rare but potentially fatal disorder associated with renal insufficiency. The condition is characterized by arteriolar calcification, thrombus formation, and distal ischemia and necrosis of the skin and subcutaneous tissue. Mortality is near 50% and is often due to ulcer-related sepsis. Hyperphosphatemia, elevated calcium (Ca) and inorganic phosphorus (Pi) product, hyperparathyroidism, use of calcium-based phosphate binders, use of Vitamin D analogs, warfarin use or Vitamin K deficiency, female gender, peritoneal dialysis (PD), and Caucasian race [1]. In 2004, Cicone et al were the first to describe the successful use of STS for the treatment of calciphylaxis [2]. We describe the successful use of intravenous STS for the treatment of CUA in two patients

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