Calciphylaxis

Calciphylaxis

Calciphylaxis (calcific uraemic arteriolopathy) is a syndrome of disseminated calcification resulting in both vascular calci fication and skin necrosis. It accounts for approximately 4% of patients undergoing surgical intervention for secondary hyperparathyroidism. It presents with expanding painful cutaneous purpuric lesions, predominantly on the e xtremities, although they can also be seen on the lower abdomen. The underlying tissue calcification within the arteriolar and small vascular walls leads to ischaemic necrosis and the development of gangrene, which in turn leads to overwhelming sepsis and death. The majority of these patients will have an elevated calcium /uni00A0×/uni00A0 phosphate product but it is not usually associated with an extremely high PTH level. The underlying aetiology remains unclear but a number of potential factors have been postulated. A reduction in the serum levels of a calcification inhibitory protein, α -Heremans–Schmid glycoprotein, 2 and abnormalities in smooth muscle cell biology in uraemic patients may play a role in the development of the syndrome. Prognosis for these patients is extremely poor, with a mortality of up to 87%. An urgent parathyroidectomy has been shown to J F Heremans , 1927–1975, Professor of Medicine, Catholic University of Louvain, Belgium. Karl Schmid , 1920–2009, Biochemist, Boston Medical Center, Boston, MA, USA. amputation in these patients. It has also been associated with an increase in median survival. Calciphylaxis

Calciphylaxis (calcific uraemic arteriolopathy) is a syndrome of disseminated calcification resulting in both vascular calci fication and skin necrosis. It accounts for approximately 4% of patients undergoing surgical intervention for secondary hyperparathyroidism. It presents with expanding painful cutaneous purpuric lesions, predominantly on the e xtremities, although they can also be seen on the lower abdomen. The underlying tissue calcification within the arteriolar and small vascular walls leads to ischaemic necrosis and the development of gangrene, which in turn leads to overwhelming sepsis and death. The majority of these patients will have an elevated calcium /uni00A0×/uni00A0 phosphate product but it is not usually associated with an extremely high PTH level. The underlying aetiology remains unclear but a number of potential factors have been postulated. A reduction in the serum levels of a calcification inhibitory protein, α -Heremans–Schmid glycoprotein, 2 and abnormalities in smooth muscle cell biology in uraemic patients may play a role in the development of the syndrome. Prognosis for these patients is extremely poor, with a mortality of up to 87%. An urgent parathyroidectomy has been shown to J F Heremans , 1927–1975, Professor of Medicine, Catholic University of Louvain, Belgium. Karl Schmid , 1920–2009, Biochemist, Boston Medical Center, Boston, MA, USA. amputation in these patients. It has also been associated with an increase in median survival.