Treatment of Calciphylaxis
The treatment of calciphylaxis requires a comprehensive multidisciplinary approach that includes correction of calcium-phosphate metabolism, parathyroidectomy for patients with severe hyperparathyroidism, sodium thiosulfate administration, wound care, and discontinuation of contributing medications. 1, 2
First-Line Management
Correction of Calcium-Phosphate Metabolism
- Normalize calcium-phosphate product (target <70 mg²/dl²) 2
- Discontinue medications that contribute to calciphylaxis:
- Warfarin (vitamin K antagonists)
- Active vitamin D supplements
- Calcium-based phosphate binders
- Iron supplements 3
Surgical Intervention
- Parathyroidectomy (subtotal or total) is indicated for patients with:
- Parathyroidectomy has shown significant improvement in wound healing (complete healing observed by 4.8 ± 2 months) 4
Pharmacological Therapy
- Sodium thiosulfate (intravenous):
- Mechanism: Displaces calcium ions from deposits to form calcium thiosulfate, which is excreted by kidneys or removed by dialysis
- Demonstrated improvements in wound healing 5
- Bisphosphonates to reduce calcium release from bone 5
- Cinacalcet for management of secondary hyperparathyroidism 5
Wound Management
- Aggressive wound debridement of necrotic tissue by vascular surgical service 4
- Pain management (calciphylaxis wounds are extremely painful)
- Skin grafting for large wounds after adequate debridement 4
- Evaluation of vascular status and revascularization if needed 4
Dialysis Optimization
- Intensification of dialysis regimen for patients with end-stage renal disease
- Use of low calcium dialysate 2
Adjunctive Therapies
- Hyperbaric oxygen therapy to promote wound healing 5
- SNF472 (hexaphosphate phytate) shows promise in clinical trials for improving wound healing and reducing mortality 2
- Iloprost infusions may help with peripheral perfusion 5
Monitoring and Expected Outcomes
- Pain improvement and initial wound healing typically occurs within 2-4 weeks of appropriate treatment
- Complete resolution may take many months
- Monitor calcium, phosphate, and PTH levels regularly
- Mortality remains high (>50% at one year) despite treatment 2
Special Considerations
- Vascular surgeons play a key role in the multidisciplinary team for wound assessment and management 4
- Skin biopsy has limited utility (variable sensitivity of 20-80%) and may worsen tissue damage; diagnosis is primarily clinical 2
- Early recognition and prompt intervention are critical to improving outcomes 2, 4
The treatment of calciphylaxis is challenging and requires coordination between nephrology, dermatology, vascular surgery, endocrinology, and wound care specialists to optimize outcomes in this devastating condition.