Survival, Risk Factors, and Effect of Treatment in 101 Patients With Calciphylaxis

McCarthy, James T.; el‐Azhary, Rokea A.; Patzelt, Michelle T.; Weaver, Amy L.; Albright, Robert C.; Bridges, Alina D.; Claus, Paul; Davis, Mark D.P.; Dillon, John; El-Zoghby, Ziad M.; Hickson, La Tonya J.; Kumar, Rajiv; McBane, Robert D.; McCarthy-Fruin, Kathleen; McEvoy, Marian T.; Pittelkow, Mark R.; Wetter, David A.; Williams, Amy W.

doi:10.1016/j.mayocp.2016.06.025

Cited by 165 publications

(188 citation statements)

References 19 publications

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“…The skin lesions are initially violaceous that progress to livedo reticularis and eventually form eschar with non-healing ulcers within days to few weeks [1,6,10]. They have a distal or sacral distribution with a propensity for areas with abundant adipose tissue [2,13]. …”

Section: Discussionmentioning

confidence: 99%

Multifactorial aetiology for non-uremic calciphylaxis: a case report

Basnet

Tachamo

Dhital

et al. 2018

Journal of Community Hospital Internal Medicine Perspectives

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Calciphylaxis is commonly associated with end-stage renal disease patients on haemodialysis. We present a rare case of calciphylaxis in a non-uremic patient. The diagnosis was made clinically and confirmed with skin biopsy showing calcification of the dermal and subcutaneous tissues in the von Kossa stain. We believe that the combination of uncontrolled diabetes mellitus, a non-functioning paraganglioma and vitamin D deficiency in a susceptible female patient was responsible for causing calciphylaxis in our patient. An index of suspicion should be maintained by clinicians for calciphylaxis even in patients without uremia.

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Section: Discussionmentioning

confidence: 99%

Multifactorial aetiology for non-uremic calciphylaxis: a case report

Basnet

Tachamo

Dhital

et al. 2018

Journal of Community Hospital Internal Medicine Perspectives

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“…In the German Calciphylaxis Registry, 10% of the total patients with calciphylaxis had either normal kidney function or had CKD not requiring renal replacement therapy [7 ■■ ]. In a recent single center report from Mayo Clinic, nearly 20% of all patients with calciphylaxis had estimated glomerular filtration rate greater than 60 ml/min [13 ■■ ]. Specific calciphylaxis incidence rates in patients with CKD or in those with normal renal function are not available; however, it is reasonable to conclude that the incidence of calciphylaxis is lower in the patients with non-ESRD compared to the patients with ESRD.…”

Section: Incidence and Prevalencementioning

confidence: 99%

“…Findings from recent large studies provide important insights into the predisposing factors for calciphylaxis [6 ■■ ,7 ■■ ,13 ■■ ,26 ■ ]. Table 1 summarizes these findings and highlights important methodological differences that may account for differences in observations from these studies.…”

Section: Risk Factorsmentioning

confidence: 99%

“…Previous and recent data regarding the efficacy of interventions including intravenous sodium thiosulfate, intralesional sodium thiosulfate, bisphophonates, vitamin K, surgical debridement, dermal regenerative template, hyperbaric oxygen, surgical parathyroidectomy, novel anticoagulants, renal transplantation, becaplermin (recombinant platelet-derived growth factor), and prostaglandins are derived from retrospective cohort studies, case series, or case reports [2,7 ■■ ,13 ■■ ,35–48]. These interventions need further investigation and at present it is best to adopt a case by case approach that takes into account risk–benefits and limitations of the currently available treatments in the management of calciphylaxis.…”

Section: Treatmentmentioning

confidence: 99%

“…The outcomes in calciphylaxis patients remain poor with mortality rate at approximately 30% at 6 months and 50% at 12 months [6 ■■ ,13 ■■ ]. Septicemia from the infected wounds is the leading cause of death.…”

Section: Prognosismentioning

confidence: 99%

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Calciphylaxis

Nigwekar¹

2017

Current Opinion in Nephrology and Hypertension

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Purpose of review Calciphylaxis remains a poorly understood vascular calcification disorder with predilection for patients with end-stage renal disease (ESRD). Recent data from large patient registries and databases have begun to provide information regarding incidence, risk factors, and outcomes in patients with calciphylaxis. Recent findings The most recent estimate places the incidence of calciphylaxis at 3.5 new cases/1000 patient-years among the patients with ESRD on chronic hemodialysis. It is possible that misdiagnosis or subclinical disease may attribute to lower than the true incidence. There is a suggestion that the incidence is higher in peritoneal dialysis patients compared with patients with hemodialysis. Recent studies have identified a number of risk factors and point to the effects of vitamin K deficiency mediated impairment in Matrix Gla Protein carboxylation as one of the likely pathogenic mechanisms. The outcomes in calciphylaxis patients remain poor with mortality approaching 30% at 6 months and 50% at 12 months. Summary The present review describes recent literature in the field of calciphylaxis. Calciphylaxis registries and specimen biorepositories promise to provide insights into the pathogenesis of calciphylaxis and will pave the way for much needed clinical trials.

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