Calciphylaxis, more appropriately termed calcific uremic arteriolopathy, is a clinical syndrome of skin necrosis due to medial calcification of the small arterioles. While this disease has been described as a distinct entity, we believe it is but a single manifestation of a complicated systemic process that leads to vascular calcification in both the intimal and medial layers of all forms of arteries in patients with chronic kidney disease. The pathophysiology of vascular calcification includes injury leading to vascular smooth muscle cell de-differentiation to osteoblast-like cells, a nidus "formation" of matrix proteins with and without apoptotic bodies, initiation and extension of mineralization, and a balance of pro-calcification factors and inhibitory calcification factors. The clinical manifestations depend upon the location of the affected artery. The rationale behind this hypothesis is discussed in this review.
- Calcific uremic arteriolopathy
- Chronic kidney disease
- Core-binding factor α-1
- Vascular calcification
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health