Calciphylaxis: A Large Case Series

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.