Management of Calciphylaxis
The management of calciphylaxis requires a multimodal approach including sodium thiosulfate, pain control, wound care, discontinuation of vitamin K antagonists, and addressing underlying mineral bone disorders, though evidence for most interventions remains limited. 1, 2
First-Line Interventions
- Discontinue vitamin K antagonists (e.g., warfarin) immediately, as they increase calciphylaxis risk up to 11-fold 2, 3
- Sodium thiosulfate (12.5-25g per session, 2-3 times weekly for 3-6 months) is widely used despite limited evidence - a recent meta-analysis did not find an association between sodium thiosulfate and wound improvement or survival 1, 2
- Non-vitamin K oral anticoagulants (reduced dose of apixaban) may be a safer alternative to warfarin in patients with kidney failure on dialysis who have atrial fibrillation with calciphylaxis 1
- Pain control is essential and should be prioritized as calciphylaxis lesions are extremely painful 1, 2
- Advanced care planning and shared decision-making are important aspects of management given the high mortality rate 1
Wound Management
- Wound care is critical and should include regular assessment of healing progress 2, 4
- Surgical debridement may be considered for infection control or intractable pain, though this requires careful patient selection 4
- Prevention of wound infection is crucial as sepsis is a leading cause of mortality 5, 6
Addressing Mineral Bone Disorders
- Parathyroidectomy should be considered for patients with significantly elevated PTH levels (>500 pg/mL) 2, 6
- Magnesium supplementation may help prevent phosphate-induced vascular calcification, though evidence is mixed 1, 2
- Monitor calcium-phosphate product and work to normalize levels 5
Monitoring and Follow-up
- Regular monitoring of C-reactive protein as it is the most helpful laboratory test reflecting inflammation in calciphylaxis diagnosis and treatment response 2, 3
- Monitor wound healing and assess for signs of infection 2, 4
Experimental Therapies
- Tissue plasminogen activator has been used as an adjunctive treatment with approximately 30% greater survival than controls in small studies, though not statistically significant and associated with bleeding risk 7
- SNF472 (hexaphosphate phytate) has shown promise in clinical trials for calciphylaxis, with improvements in wound healing and reduced hospitalizations 3
Prognosis
- Calciphylaxis carries a poor prognosis with mortality rates of 60-80%, primarily due to sepsis from infected wounds 5
- Early recognition and prompt intervention with a multidisciplinary approach may improve outcomes 8, 4
Common Pitfalls and Caveats
- Avoid skin biopsy when possible due to poor sensitivity (20-80%) and risk of traumatizing vulnerable tissue 2, 3
- Serum calcium and phosphate levels are not predictive of outcomes in calciphylaxis and cannot be reliably used for guiding therapy 3
- Vitamin D derivatives should be used cautiously as they may be a risk factor for calciphylaxis 3
- Do not delay treatment while awaiting histological confirmation, as early intervention is critical 8, 6