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Calciphylaxis: Natural history, risk factor analysis, and outcome

https://doi.org/10.1016/j.jaad.2006.08.065Get rights and content

Background

Calciphylaxis is characterized by ischemic cutaneous ulceration, high mortality, and ineffective treatment.

Methods

We conducted a retrospective study of 64 patients with calciphylaxis (including 49 dialysis patients age- and sex-matched to 98 dialysis controls).

Results

The estimated 1-year survival rate of calciphylaxis was 45.8%. Risk factors for calciphylaxis included obesity, liver disease, systemic corticosteroid use, calcium-phosphate product more than 70 mg2/dL2, and serum aluminum greater than 25 ng/mL. Survival rates were similar for 16 patients who received parathyroidectomy and 47 who did not. An estimated 1-year survival rate of 61.6% was observed for 17 patients receiving surgical debridement compared with 27.4% for the 46 who did not (P = .008).

Limitations

The study was limited by its retrospective design and there was no control group for the 15 nondialysis cases.

Conclusions

Calciphylaxis is multifactorial and usually fatal. Prevention of calciphylaxis may include correction of risk factors identified in this study. Surgical debridement was associated with improved survival, but parathyroidectomy was not.

Section snippets

Methods

The study was approved by the Institutional Review Board of Mayo Foundation and consisted of a retrospective review of the medical records of 64 patients in whom calciphylaxis was diagnosed at Mayo Clinic, Rochester, Minnesota, over an 11-year period (1992 to 2002). This included 16 patients in the previous study of Kang et al.1 Of the 64 patients, 49 (77%) were receiving dialysis for end-stage renal failure (“dialysis patients”). The remaining 15 patients were designated “nondialysis

Results

Follow-up information was available for 63 of the 64 patients with calciphylaxis, of whom 51 (81%) had died by the completion of the study. The median survival from the date of diagnosis for these 63 patients was 2.64 months (range, 0-7 years). Overall and group-specific survival curves are presented in Fig 1. At the time of death, calciphylaxis-related cutaneous ulceration was present in 34 patients (67%), and the estimated cause-specific survival rate at 1 year was 45.8%. Sepsis was a

Discussion

In 1961, Selye, Gentile, and Prioreschi2 defined “calciphylaxis” as a systemic hypersensitivity reaction analogous to an allergic reaction (anaphylaxis). In experiments on rodents, Selye and coworkers induced calcification of various organs (including the skin) after animals had been “sensitized” with one of several agents they referred to as “calcifiers” (eg, dihydrotachysterol, vitamin D2, vitamin D3, and parathyroid hormone), followed by exposure to a “challenger” (eg, metallic salts such as

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  • Cited by (0)

    Funding sources: None.

    Conflicts of interest: None identified.

    Visiting Medical Student, Mayo Clinic College of Medicine.

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