Calcific uremic arteriolopathy (calciphylaxis): Case series and review of clinical features and terminology

Michael R. Rickels, Jacqueline M. Junkins-Hopkins, Thomas S. Metkus, Nayyar Iqbal

Research output: Contribution to journalReview articlepeer-review

Abstract

We present 3 cases of calciphylaxis, better termed calcific uremic arteriolopathy (CUA), which illustrate typical and atypical presentations of this disorder to discuss its classification, pathophysiology, and treatment based on a review of the literature. Of the 3 patients presenting with biopsy-proven CUA, 1 patient with proximal lesions expired in the setting of upper gastrointestinal bleeding and superinfection of ulcers, a second patient had an extensive distal ulcerative lesion that failed to heal and necessitated lower-extremity amputation, and a third patient with multiple small distal lesions experienced complete resolution of the ulcers. The classification, pathophysiology, and treatment of CUA are detailed here. CUA is a heterogeneous syndrome which typically follows a progressive course leading to loss of life or limb. Diagnosis requires a high index of suspicion and low threshold for biopsy in at-risk patients. Parathyroidectomy is appropriate to rapidly lower the calcium-phosphorus product in the setting of marked hyperparathyroidism; additional therapy includes the avoidance of purported precipitants, medical lowering of the serum calcium and phosphorus, and promotion of wound healing with hyperbaric oxygen therapy.

Original languageEnglish (US)
Pages (from-to)57-62
Number of pages6
JournalEndocrinologist
Volume17
Issue number1
DOIs
StatePublished - Jan 1 2007
Externally publishedYes

Keywords

  • Calcium phosphorus product
  • Chronic kidney disease
  • Cutaneous ulceration
  • Hyperparathyroidism

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

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