Clinical Nephrology – Epidemiology – Clinical Trials

Kidney International (2002) 61, 2210–2217; doi:10.1046/j.1523-1755.2002.00375.x

Calciphylaxis is usually non-ulcerating: Risk factors, outcome and therapy

Adrian Fine and James Zacharias

Section of Nephrology, Department of Medicine, University of Manitoba, Winnipeg, Canada

Correspondence: Dr. Adrian Fine, Room BG007, St. Boniface General Hospital, 409 Tache Avenue, Winnipeg, Manitoba, Canada R2H 2A6. E-mail: afine@sbgh.mb.ca

Received 18 September 2001; Revised 28 January 2002; Accepted 29 January 2002.

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Abstract

Calciphylaxis is usually non-ulcerating: Risk factors, outcome and therapy.

Background

 

Calciphylaxis, historically considered rare, seems to be increasing in frequency. In our single center, 36 new cases have accumulated in seven years. The majority of these cases were non-ulcerating, which we believe to be early disease, in contradistinction to the vast majority of published cases that presented with ulcers.

Methods

 

Prospective data were collected on all patients with calciphylaxis. As well, a case control study, with two controls per patient, was performed on patients presenting with non-ulcerating plaques.

Results

 

The incidence of calciphylaxis in dialysis patients increased with a rate of 4.5/100 patient-years in the past three years. Eighty percent of cases presented with non-ulcerating subcutaneous plaques in the calves, easily confused with cellulitis. In those patients presenting with plaques only, the mortality rate was 33% at six months. Once ulceration develops, the mortality rate increased to above 80%. Bone scan was positive in 97% of patients. Steroid therapy appeared to be beneficial in some patients. Peritoneal dialysis, female sex and diabetes were risk factors. In the case control study of patients presenting with plaques only, serum phosphate (OR 2.6; 95% CI 1.05 to 6.45, P = 0.038) and Ca times P product (OR 1.46; 95% CI 1.02 to 20, P = 0.038) predicted the disease, as did being on calcium salts + vitamin D (OR 4.05; 95% CI 1.14 to 14.5, P = 0.03).

Conclusions

 

Calciphylaxis is no longer rare. It is usually nonulcerating and can be diagnosed clinically in all patients. These patients have a high mortality, especially once ulceration occurs. Calcium salts plus vitamin D, as well as serum Ca times P product and high serum P increase the chance of the diseases. Therefore, the disease may be preventable. Steroids may be of benefit to some patients.

Keywords:

hyperphosphatemia, calcium salts, dialysis, end-stage renal disease, kidney calciphylaxis, phosphate-lowering therapy

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