Calciphylaxis: A Large Case Series

November 3, 2016

Calciphylaxis: A Large Case Series

  1. Allan S. Brett, MD

This disorder has a poor prognosis; thrombophilias frequently are associated with it.

  1. Allan S. Brett, MD
Calciphylaxis Images
Image A: Hand radiograph showing severely calcified arteries and loss of soft tissue at the tips of the index and middle fingers (visible in the image, along with an intravenous catheter at the wrist). The results of skin biopsy were consistent with calciphylaxis. Image B: Calciphylaxis on the skin of a patient's flank. Image A: Reprinted from Li JZ and Huen W. N Engl J Med 2007; 357:1326; Image B: Reprinted from Bazari H et al. N Engl J Med 2007; 356:1049 (Figure 2).
Calciphylaxis Images

Image A: Hand radiograph showing severely calcified arteries and loss of soft tissue at the tips of the index and middle fingers (visible in the image, along with an intravenous catheter at the wrist). The results of skin biopsy were consistent with calciphylaxis. Image B: Calciphylaxis on the skin of a patient's flank.

Image A: Reprinted from Li JZ and Huen W. N Engl J Med 2007; 357:1326; Image B: Reprinted from Bazari H et al. N Engl J Med 2007; 356:1049 (Figure 2).

Calciphylaxis (also known as calcific uremic arteriolopathy, because it is seen mostly in patients with end-stage renal disease [ESRD]) is an uncommon disorder characterized by painful necrotic lesions involving subcutaneous adipose tissue and overlying skin (see Figure). Mayo Clinic researchers present this retrospective case series of 101 patients, who were seen during 15 years.

About 60% of patients had ESRD, 20% had lesser stages of chronic kidney disease, and 20% did not have renal impairment. The median time to death after diagnosis of calciphylaxis was 4 months; estimated 1-year survival was 37%. Treatments included sodium thiosulfate, tissue-plasminogen activator, hyperbaric oxygen, surgical debridement, and parathyroidectomy. In analyses not adjusted for confounders, surgical debridement and parathyroidectomy were associated with somewhat better survival.

The researchers document a high prevalence of thrombophilia in this cohort. Among 55 patients who had extensive thrombophilia testing, 33 had a severe thrombophilia (most commonly lupus anticoagulant and antithrombin deficiency). Half of the 55 patients were taking warfarin, a previously described risk factor for calciphylaxis. Warfarin reduces levels of protein C and S, but severe thrombophilia was documented in 23 of 27 patients who were not taking warfarin at the time of testing.

Comment

This case series illustrates the seriousness of calciphylaxis; anecdotal support exists for the various treatments noted above, but none has been evaluated rigorously. The intriguing data on thrombophilias suggest that a tendency to thrombosis, superimposed on the underlying vascular calcification that occurs commonly in chronic kidney disease, is an important contributor to this disorder.

Editor Disclosures at Time of Publication

  • Disclosures for Allan S. Brett, MD at time of publication Nothing to disclose

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