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| {{Calciphylaxis}} | | {{Calciphylaxis}} |
| {{CMG}} | | {{CMG}} |
| ==Overview==
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| '''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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| ==Pathophysiology== | | ==[[Calciphylaxis overview|Overview]]== |
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| Calciphylaxis is characterised by | | ==[[Calciphylaxis historical perspective|Historical Perspective]]== |
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| # 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
| | ==[[Calciphylaxis pathophysiology|Pathophysiology]]== |
| # 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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| ==Causes== | | ==[[Calciphylaxis causes|Causes]]== |
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| 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.
| | ==[[Calciphylaxis differential diagnosis|Differentiating Calciphylaxis from other Diseases]]== |
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| ==Differentiating Calciphylaxis from other Diseases== | | ==[[Calciphylaxis epidemiology and demographics|Epidemiology and Demographics]]== |
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| 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. | | ==[[Calciphylaxis risk factors|Risk Factors]]== |
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| ==Risk Factors== | | ==[[Calciphylaxis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| 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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| ==Natural History, Complications and Prognosis ==
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| Severe forms of calciphylaxis may cause diastolic [[heart failure]] from cardiac calcification, called ''heart of stone''.<ref>[http://www.mayoclinicproceedings.com/pdf%2F8103%2F8103mi1.pdf Heart of Stone] - CINDY W. T OM, MD,ANDDEEPAKR. TALREJA, MD. Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, Minn</ref>
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| 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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| ==Diagnosis== | | ==Diagnosis== |
| | | [[Calciphylaxis history and symptoms|History and Symptoms]] | [[Calciphylaxis physical examination|Physical Examination]] | [[Calciphylaxis biopsy|Biopsy]] |
| There is no diagnostic test for calciphylaxis. The diagnosis is a clinical one. The characteristic lesions are the ischaemic skin lesions (usually with areas of skin necrosis). They should alert the physician or nurse.
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| The necrotic skin lesions (i.e., the dying or already dead skin areas) typically appear as violaceous (dark bluish purple) lesions and/or completely black leathery lesions. They can be extensive.
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| The suspected diagnosis can be confirmed by a skin [[biopsy]]. It shows arterial calcification and occlusion in the absence of [[vasculitis]].
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| ==Treatment== | | ==Treatment== |
| | [[Calciphylaxis medical therapy|Medical Therapy]] | [[Calciphylaxis surgery|Surgery]] | [[Calciphylaxis prevention|Prevention]] | [[Calciphylaxis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Calciphylaxis future or investigational therapies|Future or Investigational Therapies]] |
| | ==Case Studies== |
| | [[Calciphylaxis case study one|Case #1]] |
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| The optimal treatment is prevention. Rigorous and continuous control of phosphate and calcium balance most probably will avoid the metabolic changes which may lead to calciphylaxis.
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| There is no specific treatment. Of the treatments that exist, none is internationally recognised as the standard of care. An acceptable treatment could include:
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| *[[Dialysis]] (the number of sessions may be increased)
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| *Intensive wound care
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| *Adequate pain control
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| *Correction of the underlying plasma calcium and phosphorus abnormalities (lowering the Ca x P product below 55 mg2/dL2)
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| *Avoiding (further) local tissue trauma (including avoiding all subcutaneous injections, and all not-absolutely-necessary infusions and transfusions)
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| *Urgent parathyroidectomy: The efficacy of this measure remains uncertain although calciphylaxis is associated with frank hyperparathyroidism. Urgent parathyroidectomy may benefit those patients who have uncontrollable plasma calcium and phosphorus concentrations despite dialysis. Also, [[cinacalcet]] can be used and may serve as an alternative to parathyroidectomy. The trade name of [[cinacalcet]] is [[Sensipar]] or [[Mimpara]].
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| *Patients who receive [[kidney transplant]]s also receive [[immunosuppression]]. Considering lowering the dose of or discontinuing the use of immunosuppressive drugs in renal transplant patients who continue to have persistent or progressive calciphylactic skin lesions can contribute to an acceptable treatment of calciphylaxis.
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| ==Refernces==
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| {{reflist|2}}
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| ==External links== | | ==External links== |
| * [http://content.nejm.org/cgi/content/full/357/13/1326?query=TOC "Images in Clinical Medicine: Calciphylaxis with Arterial Calcification", from "The New England Journal of Medicine] | | * [http://content.nejm.org/cgi/content/full/357/13/1326?query=TOC "Images in Clinical Medicine: Calciphylaxis with Arterial Calcification", from "The New England Journal of Medicine] |