Calciphylaxis
Calciphylaxis, also called calcemic uremic arteriopathy, is a form of vascular calcification that produces extensive calcification of the skin, muscles, and subcutaneous tissue, leading to ischemic ulceration and necrosis, most often in patients with end-stage renal disease. There is no single diagnostic test; management centers on normalizing serum calcium and phosphate, increasing the intensity of dialysis, non-calcium phosphate binders and calcimimetic therapy, and aggressive pain control and wound care.
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What this article covers
- Overview
Definition: calciphylaxis, also called calcemic uremic arteriopathy, is a form of vascular calcification characterized by extensive calcification of the skin, muscles, and subcutaneous tissues, leading to ulceration and necrosis.
- Epidemiology
Occurs predominantly in end-stage renal disease: calciphylaxis is described as calcemic uremic arteriopathy, reflecting its strong association with uremia and chronic kidney disease, and it is most…
- Risk factors
End-stage renal disease, particularly with long-term hemodialysis: the chronic disordered calcium and phosphorus metabolism of CKD is the dominant risk factor for calciphylaxis.
- Pathophysiology
CKD-mineral and bone disorder (CKD-MBD) underlies most cases: CKD results in disordered calcium and phosphorus metabolism, producing a broad spectrum of disorders including hyperparathyroidism…
- Clinical features
Extensive calcification of the skin, muscles, and subcutaneous tissue leading to ulceration and necrosis is the defining feature: lesions typically evolve from painful, poorly demarcated skin changes…
- Differential diagnosis
Purpura fulminans: rapidly progressive hemorrhagic skin necrosis from disseminated intravascular coagulation in a septic or asplenic patient, unlike the chronic renal and mineral-disease context and…
- Diagnosis
There is no specific diagnostic test for calciphylaxis; the diagnosis is clinical, supported by biopsy in a patient with the characteristic risk factors and lesion pattern: severely painful, poorly…
- Management
First, correct the metabolic drivers by normalizing serum calcium and phosphate levels, since treatment is aimed at normalizing calcium, phosphate, and PTH while minimizing the risks of therapy.
- Complications
Painful, non-healing ulceration and necrosis: extensive calcification of the skin, muscles, and subcutaneous tissue leads directly to ulceration and necrosis that can be severely painful and slow to heal.
- Prognosis
No specific treatment is established, so outcomes depend on how effectively the mineral and bone disorder (calcium, phosphate, PTH) can be normalized and dialysis intensified.
- Prevention
Monitor calcium, phosphorus, PTH, and calcitriol regularly in patients with CKD, since ongoing surveillance of the mineral axis is the basis for detecting and treating hyperparathyroidism before…
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