Calciphylaxis

Management Strategy

Calciphylaxis has a mortality of up to 80%, which underscores the importance of early diagnosis via skin biopsy and aggressive treatment. The pillars of treatment for calciphylaxis are monitoring for infection, aggressive wound care, calcium chelation, and addressing underlying metabolic imbalance.

Sepsis is the leading cause of death in calciphylaxis, which necessitates consistent surveillance for wound infection including consideration of tissue and wound cultures, monitoring for systemic signs of infection , and prompt initiation of antibiotics when necessary . Early and aggressive debridement of necrotic tissue is important to remove a potential nidus of infection.

Intravenous sodium thiosulfate (STS) is used to remove calcium deposits by chelation. STS is currently considered a standard of care. Intralesional STS has also been used.

Addressing the underlying metabolic dysregulation is critical. Serum calcium should be decreased by discontinuing calcium and vitamin D supplementation . Hyperphosphatemia must be reduced with dietary phosphorus restriction and usage of non-calcium–based phosphate binders. Discontinuation of vitamin K antagonists (VKA) such as warfarin is critical to promote vitamin K–dependent production of matrix Gla protein, a potent inhibitor of arterial calcification. In renal failure, the use of low-calcium dialysate to solubilize calcium into the intravascular space is helpful. Bisphosphonates can be used to decrease arterial calcification.

Hyperparathyroidism may be treated with cinacalcet with continued surveillance of parathyroid hormone levels. Parathyroidectomy is an option for recalcitrant hyperparathyroidism, but risks and benefits of surgery must be weighed.

Patients should be evaluated for hypercoagulability and autoimmune conditions. Hyperbaric oxygen therapy and negative pressure wound therapy also have potential utility.

Multi-interventional therapy is important in calciphylaxis management.