Calciphylaxis: Symptoms, Causes, Diagnosis & Treatment

Calciphylaxis (calcific uremic arteriolopathy) is a rare, life-threatening syndrome of small-vessel medial calcification, thrombosis, and skin necrosis. It predominantly affects patients with end-stage kidney disease (ESKD) on dialysis, but also occurs in earlier CKD, post-transplant, and non-uremic settings (malignancy, autoimmune, primary hyperparathyroidism, warfarin use).

Clinical Features

• Skin Lesions: Livedo reticularis → painful violaceous plaques → stellate ulcerations with black eschars. Typically on thighs, abdomen, buttocks; can occur anywhere.
• Systemic: Sepsis from superinfection (major cause of death), multiorgan failure.

Key Laboratory Findings

• Elevated calcium × phosphate product (>55 mg²/dL²).
• Hyperphosphatemia, hypercalcemia (or normal Ca²⁺), elevated PTH (secondary/tertiary hyperparathyroidism).
• Low 25(OH) vitamin D, elevated FGF-23.

Diagnosis

• Clinical presentation + skin biopsy: Medial arterial calcification, intimal hyperplasia, thrombosis, no vasculitis (leukocytoclastic).
• Imaging (bone scan, CT) may show vascular calcification.
• Exclude mimics: vasculitis, atheroemboli, necrotizing fasciitis, warfarin-induced skin necrosis.

Treatment (Multidisciplinary: Nephrology, Dermatology, Wound Care, Surgery)

• Wound Care: Sharp debridement (if stable), antimicrobial dressings, negative pressure therapy (VAC).
• Infection Control: Broad-spectrum antibiotics for sepsis; targeted for wound cultures.
• Mineral Metabolism Correction: Non-calcium phosphate binders (sevelamer, lanthanum, sucroferric oxyhydroxide), cinacalcet (calcimimetic), low-calcium dialysate (2.0–2.5 mEq/L), parathyroidectomy if severe HPT.
• Sodium Thiosulfate: IV (25 g 3×/week post-dialysis) or topical — chelates calcium, antioxidant; evidence evolving (case series, small RCTs).
• Vitamin K2 (Menaquinone-7): Activates matrix Gla protein (endogenous calcification inhibitor); promising adjunct.
• Other: Hyperbaric oxygen, bisphosphonates, anticoagulation (LMWH) — case reports/series.

Prognosis

1-year mortality 45–80%, primarily from sepsis. Early recognition, aggressive mineral metabolism control, meticulous wound care, and multidisciplinary coordination improve outcomes.