Calciphylaxis

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Calciphylaxis
Calciphylaxis.png
Calciphylaxis on the abdomen of a patient with end stage renal disease. Markings are in cm.
Classification and external resources
ICD-9275.49
DiseasesDB1897
eMedicinederm/555
MeSHD002115
 
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Calciphylaxis
Calciphylaxis.png
Calciphylaxis on the abdomen of a patient with end stage renal disease. Markings are in cm.
Classification and external resources
ICD-9275.49
DiseasesDB1897
eMedicinederm/555
MeSHD002115

Calciphylaxis, or calcific uremic arteriolopathy (CUA), is a syndrome of vascular calcification, thrombosis and skin necrosis. It is seen almost exclusively in patients with Stage 5 chronic kidney disease. It results in chronic non-healing wounds and is usually fatal. Calciphylaxis is a rare but serious disease.

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

Signs and symptoms[edit]

Calciphylaxis is characterized by the following:

  1. systemic medial calcification of the arteries, i.e. calcification of tunica media. Unlike other forms of vascular calcifications (e.g., 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 ischemia (including skin ischemia and, hence, skin necrosis).

Heart of Stone[edit]

Severe forms of calciphylaxis may cause diastolic heart failure from cardiac calcification, called heart of stone.[1]

Cause[edit]

The cause is not known. It does not seem to be an immune type reaction. In other words, calciphylaxis is not a hypersensitivity reaction (i.e., allergic reaction) leading to sudden local calcification. Clearly, additional factors are involved in calciphylaxis. It is also known as calcific uremic arteriolopathy; however, the disease is not limited to patients with kidney failure.

Diagnosis[edit]

There is no diagnostic test for calciphylaxis. The diagnosis is a clinical one. The characteristic lesions are the ischemic skin lesions (usually with areas of skin necrosis). 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. The suspected diagnosis can be supported by a skin biopsy. It shows arterial calcification and occlusion in the absence of vasculitis. Sometimes the bone scintigraphy can show increased tracer accumulation in the soft tissues.[2] In certain patients, anti-nuclear antibody may play a role.[3]

Treatment[edit]

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.

There is no specific treatment. Of the treatments that exist, none is internationally recognised as the standard of care. An acceptable treatment could include:

Epidemiology[edit]

Calciphylaxis most commonly occurs in patients with end-stage renal disease who are on hemodialysis or who have recently received a renal (kidney) transplant. Yet calciphylaxis does not occur only in end-stage renal disease patients. When reported in patients without end-stage renal disease, it is called non-uremic calciphylaxis by Nigwekar et al.[5] Non-uremic calciphylaxis has been observed in patients with primary hyperparathyroidism, breast cancer (treated with chemotherapy), liver cirrhosis (due to alcohol abuse), cholangiocarcinoma, Crohn's disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE).

Prognosis[edit]

Unfortunately, response to treatment is not guaranteed. Also, the necrotic skin areas may get infected, and this then may lead to sepsis (i.e. infection of blood with bacteria; sepsis can be life-threatening) in some patients. Overall, the clinical prognosis remains poor.

References[edit]

  1. ^ Heart of Stone - CINDY W. T OM, MD,ANDDEEPAKR. TALREJA, MD. Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, Minn
  2. ^ Araya CE, Fennell RS, Neiberger RE, Dharnidharka VR (2006). "Sodium thiosulfate treatment for calcific uremic arteriolopathy in children and young adults". Clin J Am Soc Nephrol 1 (6): 1161–6. doi:10.2215/CJN.01520506. PMID 17699342. 
  3. ^ Anti-nuclear antibody: a potential predictor of calciphylaxis in non-dialysis patients. Rashid RM, Hauck M, Lasley M. J Eur Acad Dermatol Venereol. 2008 Nov;22(10):1247-8. Epub 2008 Apr 15.
  4. ^ Edsell ME, Bailey M, Joe K, Millar I (2008). "Hyperbaric oxygen therapy in the treatment of skin ulcers due to calcific uraemic arteriolopathy: experience from an Australian hyperbaric unit.". Diving and Hyperbaric Medicine (South Pacific Underwater Medicine Society) 38 (3): 139–44. Retrieved 2013-04-02. 
  5. ^ Nigwekar SU, Wolf M, Sterns RH, Hix JK. Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol. 2008 Jul;3(4):1139-43. Epub 2008 Apr16

External links[edit]