Calciphylaxis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Calciphylaxis is a syndrome of vascular calcification and skin necrosis. It is seen exclusively in patients with end stage renal disease. It results in chronic non-healing wounds and requires parathyroidectomy and hyperbaric therapy. Calciphylaxis is a rare but serious disease.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Calciphylaxis is characterised by

1. systemic medial calcification of the arteries, i.e. calcification of tunica media. Unlike other forms of vascular calcifications (eg, intimal, medial, valvular), calciphylaxis is characterised also by
2. small vessel mural calcification with or without endovascular fibrosis, extravascular calcification and vascular thrombosis, leading to tissue ischaemia (including skin ischaemia and, hence, skin necrosis).

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

• The cause is not known. It does not seem to be an immune type reaction. In other words, calciphylaxis is not only a hypersensitivity reaction (= allergic reaction) leading to sudden local calcification. Clearly, additional factors are involved in calciphylaxis. It possibly could be described as calcific uraemic arteriolopathy.
• Secondary hyperparathyroidism may sometimes cause calciphylaxis.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Calciphylaxis is one type of extraskeletal calcification. Similar extraskeletal calcifications are observed in some patients with hypercalcaemic states, including patients with milk alkali syndrome, sarcoidosis, primary hyperparathyroidism, and hypervitaminosis D.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Calciphylaxis most commonly occurs in patients with end-stage renal disease who are on hemodialysis or who have recently received a renal transplant (kidney transplant). Yet, calciphylaxis does not occur only in end-stage renal disease patients. It also has been reported in patients with breast cancer (treated with chemotherapy), liver cirrhosis (due to alcohol abuse), cholangiocarcinoma, Crohn’s disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE) (including SLE patients with or without chronic renal disease).

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Severe forms of calciphylaxis may cause diastolic heart failure from cardiac calcification, called heart of stone.^[1] Unfortunately, response to treatment is not guaranteed. Also, the necrotic skin areas may get infected, and this then may lead to sepsis (ie, infection of blood with bacteria; sepsis can be life-threatening) in some patients. Overall, the clinical prognosis remains poor.

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