Calciphylaxis
| Calciphylaxis | ||
| DiseasesDB | 1897 | |
|---|---|---|
| MeSH | C18.452.174.130.186 | |
|
Calciphylaxis Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Calciphylaxis On the Web |
|
American Roentgen Ray Society Images of Calciphylaxis |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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.
Pathophysiology
Calciphylaxis is characterised by
- 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
- 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).
Causes
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.
Differentiating Calciphylaxis from other Diseases
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.
Risk Factors
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).
Natural History, Complications and Prognosis
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.
Diagnosis
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.
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 confirmed by a skin biopsy. It shows arterial calcification and occlusion in the absence of vasculitis.
Treatment
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:
- Dialysis (the number of sessions may be increased)
- Intensive wound care
- Adequate pain control
- Correction of the underlying plasma calcium and phosphorus abnormalities (lowering the Ca x P product below 55 mg2/dL2)
- Avoiding (further) local tissue trauma (including avoiding all subcutaneous injections, and all not-absolutely-necessary infusions and transfusions)
- 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.
- Patients who receive kidney transplants 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.
Refernces
- ↑ Heart of Stone - CINDY W. T OM, MD,ANDDEEPAKR. TALREJA, MD. Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, Minn