Long-term outcomes in patients with calciphylaxis from hyperparathyroidism

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.