This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Duffy, A. Articles by Chen, H. Search for Related Content PubMed PubMed Citation Articles by Duffy, A. Articles by Chen, H.

Long-Term Outcomes in Patients With Calciphylaxis From Hyperparathyroidism

Section of Endocrine Surgery, Department of Surgery and Department of Pathology, University of Wisconsin-Madison, H4/750 Clinical Science Center, 600 Highland Avenue, Madison, Wisconsin 53792-7375

Correspondence: Address correspondence and reprint requests to: Herbert Chen, MD, FACS; E-mail: chen{at}surgery.wisc.edu.

Background: Calciphylaxis is a rare condition associated with chronic renal failure and entails a very poor prognosis. Pathogenesis is poorly understood but involves abnormalities in calcium and phosphorus metabolism that lead to vascular and extravascular calcification. Patients present with painful ulcerating plaques that progress to gangrenous wounds. Parathyroidectomy has been advanced as a life-saving intervention in these patients, but long-term results with wound healing and survival after parathyroidectomy are not well described.

Methods: Between January 1987 and October 2003, 15 patients with biopsy-confirmed calciphylaxis were treated at the University of Wisconsin. Of these 15 patients, 9 were treated with medical therapy (bisphosphonates and phosphate binders), whereas 6 underwent parathyroidectomy. The medical records were reviewed, and patients or relatives were interviewed. Survival was determined by Kaplan-Meier analysis.

Results: Four patients underwent subtotal parathyroidectomy, and two patients underwent total parathyroidectomy. All had reductions in the intact parathyroid hormone level (mean ± SD, 25.2 ± 4.5 pg/mL). Whereas all six patients treated with parathyroidectomy had partial/complete wound healing, only two of nine in the medical group had any improvements in the skin lesions (P = .006). With up to 80 months of follow-up, patients who underwent parathyroidectomy had a longer median survival compared with those who did not have surgery (39 vs. 3 months; P = .017).

Conclusions: On the basis of our long-term follow-up of this patient population, subtotal or total parathyroidectomy was associated with long-term survival and was more likely to promote healing if performed earlier in the course of disease. Therefore, patients with calciphylaxis from secondary hyperparathyroidism should be referred promptly for potential parathyroidectomy.

Key Words: Calciphylaxis • Secondary hyperparathyroidism • Calcific uremic arteriolopathy • Parathyroidectomy • Wound care

This article has been cited by other articles:

				C. B. Raymond and L. D. Wazny Sodium thiosulfate, bisphosphonates, and cinacalcet for treatment of calciphylaxis Am. J. Health Syst. Pharm., August 1, 2008; 65(15): 1419 - 1429. [Abstract] [Full Text] [PDF]

				U. Wollina, C. Helm, G. Hansel, A. Koch, J. Schonlebe, G. Haroske, and E. Kostler Deep Ulcer Shaving Combined With Split-Skin Transplantation in Distal Calciphylaxis International Journal of Lower Extremity Wounds, June 1, 2008; 7(2): 102 - 107. [Abstract] [PDF]