Although there is no cure for calciphylaxis, management typically involves a multidisciplinary approach under the expertise of a dermatologist, nephrologist, wound care specialist, and pain and palliative care specialist.
Research is underway to explore definitive treatment options for this condition.
What is calciphylaxis?
Calciphylaxis, also called calcific uremic arteriolopathy or calcific vasculopathy, is a serious condition that reduces blood supply to the skin and fatty tissue due to a buildup of calcium in the walls of blood vessels.
It is an uncommon condition that is predominantly seen in people with advanced kidney diseases. About 1% of patients undergoing renal dialysis get calciphylaxis every year.
Calciphylaxis is generally quite lethal, with about 80% of affected individuals dying within several months of onset. Most patients with calciphylaxis present with painful skin abnormalities, such as deep painful lumps, ulcers, non-healing wounds, and skin discoloration. Death generally results due to wound infection and sepsis.
What causes calciphylaxis?
The exact cause of calciphylaxis is not well understood. Studies suggest that the main cause of the condition is a blockage of the small blood vessels in the skin by a blood clot. These blood clots are believed to be caused by calcium deposition (calcification) within the blood vessel walls.
Calciphylaxis is generally seen in people with chronic kidney disease (calcific uremic arteriolopathy), but it may also be seen in people with normal kidney function (non-uremic calciphylaxis), such as those with:
- Primary hyperparathyroidism
- Diabetes mellitus
- Alcoholic liver disease
- Connective tissue disease
What are current treatment options for calciphylaxis?
Treatment aims to minimize symptoms and disease complications, with approaches including:
- Managing aggravating factors through discontinuation of iron therapy, calcium supplementation, and vitamin D supplementation (Warfarin may be discontinued as well).
- Pain management by using opioid analgesics.
- Wound care by using proper dressings and chemical debridement agents:
- Negative pressure wound therapy may be administered.
- Infected wounds may be treated with antimicrobial therapy.
- Surgical debridement of the wound may be needed.
- Vacuum-assisted closure devices have been successful in several cases of calciphylaxis after extensive debridement and before skin grafting.
- Management of calcium, phosphorus, and parathyroid hormone abnormalities, which may involve the use of certain medications, such as sevelamer carbonate, lanthanum carbonate. and cinacalcet. Surgical removal of the parathyroid gland (parathyroidectomy) may be done in refractory cases.
- Dialysis optimization by adjusting the duration or frequency of hemodialysis.
- Trial of sodium thiosulfate (STS) may be given for at least 3-4 weeks. STS is a type of salt that may help because of its antioxidant properties and vasodilatory (widening of blood vessels) effects. It is an off-label treatment that has shown a good response in many patients.
- Hyperbaric oxygen therapy, which typically involves administering oxygen at high pressure (around 2.5 atm) or with a high-flow rate (10-15 L per minute), which is about 90 minutes per day for 25 sessions.
- Low-dose tissue plasminogen activator (TPA), which may be administered to dissolve blood clots in the cutaneous blood vessels (tiny blood vessels of the skin).
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Nunley JR. Calciphylaxis. Medscape. https://emedicine.medscape.com/article/1095481-overview
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