Abstract
Purpose
Calciphylaxis or Calcific Uraemic Arteriolopathy (CUA) is a rare systemic disorder associated with endstage renal disease and carrying a very poor prognosis and high mortality. It is characterized by skin necrosis and gangrene secondary to thrombosis and calcification of dermo-epidermal arterioles. Pathogenetic mechanisms inducing calciphylaxis are for the most part unknown.
Methods
We report a case of calciphylaxis in a patient on long-term haemodialysis with severe secondary hyperparathyroidism. Despite therapies for hyperparathyroidism, the injury healed only after parathyroidectomy.
Results
Calciphylaxis is an obliterative vasculopathy. The role of severe hyperparathyroidism in the pathogenesis of calciphylaxis is not well understood. A unique feature is the phenotypic differentiation of smooth muscle cells into osteoid-like cells. The vascular smooth muscle cells, exposed to high inorganic phosphate levels, could be transformed into osteoblast-like cells in patients undergoing haemodialysis with a poor control of phosphate intake.
Conclusions
The uraemic condition contributes to vascular smooth muscle cell apoptosis and differentiation into bone forming osteoblast-like cells resulting in medial calcification. Nowadays no effective treatments are available for the successful management of calciphylaxis.
Get full access to this article
View all access options for this article.