Calciphylaxis: Causes, Symptoms, Diagnosis, Treatment, and Prevention
A rare and serious condition, capillary phylaxis affects the skin and blood vessels. When calcium deposits form in small blood vessels in the skin and fat tissue, they narrow and block blood flow, resulting in skin ulcers, tissue death, infection, and severe pain. Internal organs such as the eyes, lungs, brain, muscles, and intestines can also be affected, but this is a less common occurrence.
Calciphylaxis is most common in people with advanced kidney disease, especially those on dialysis. However, people with normal kidney function or other risk factors, such as obesity, diabetes, liver disease, cancer, or certain medications, can also develop it.
Unless properly treated, calciphylaxis can result in serious complications such as sepsis, organ failure, and death.
Causes and Risk Factors
A disruption in the balance of minerals and hormones regulating calcium and phosphate levels in the body is suspected to be the cause of calciphylaxis, but the exact cause is not yet known. Vitamin D, parathyroid hormone, and calciumimimetics are some of these substances. Calcium accumulates in blood vessels and tissues when these substances are abnormal, reducing blood flow and oxygen delivery by forming hard deposits.
Some of the risk factors that can contribute to calciphylaxis include:
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Advanced kidney disease or end-stage kidney disease (ESKD)
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Dialysis treatment
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Obesity
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Diabetes
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Liver disease
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Cancer
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Inflammatory bowel disease
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Primary hyperparathyroidism
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Autoimmune conditions
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Protein C and protein S deficiency
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Medications such as warfarin, corticosteroids, iron, calcium supplements, or phosphate binders
Symptoms and Signs
The symptoms of calciphylaxis vary depending on the location and extent of the affected blood vessels and tissues. The most common symptoms include:
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Skin lesions that start as red or purple patches and progress to open ulcers with black-brown crusts
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Lesions that are usually located on areas with high fat content, such as the thighs, buttocks, abdomen, and breasts
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Lesions that are extremely painful and sensitive to touch, pressure, or temperature
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Lesions that are slow to heal and prone to infection and bleeding
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Hypercalcemia (high levels of calcium in the blood)
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Weakness
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Fatigue
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Cramps
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Body aches
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Depression
If calciphylaxis affects the internal organs, it can cause additional symptoms such as:
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Vision loss or blindness
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Shortness of breath or difficulty breathing
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Chest pain or heart attack
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Stroke or seizures
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Muscle weakness or paralysis
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Abdominal pain or intestinal bleeding
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Blood in the urine or kidney failure
Diagnosis and Tests
An evaluation of the symptoms and a physical examination are the first steps in diagnosing calciphylaxis. The doctor may order some tests to confirm the diagnosis. These tests may include:
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Skin biopsy: A small sample of skin tissue is taken from the affected area and examined under a microscope for signs of calciphylaxis, such as calcium deposits, blood clots, and tissue damage.
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Testing of blood samples for calcium, phosphate, PTH, vitamin D, and other substances that influence a person's mineral and hormone balance is done. Infections, inflammation, and blood clot disorders can also be detected with blood tests.
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Imaging studies: X-rays, ultrasound, or CT scans can show the presence and extent of calcium deposits in the blood vessels and tissues. They can also detect any damage or complications in the internal organs.
Treatment and Management
A multidisciplinary approach is necessary to treat calciphylaxis, which involves nephrologists, dermatologists, endocrinologists, surgeons, wound care nurses, and pain management doctors. The main goals of treatment are:
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Restore blood flow and oxygen delivery to the affected tissues
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Reduce or prevent further calcium deposits
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Heal the skin ulcers and prevent infection
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Control pain and improve quality of life
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Address the underlying causes and risk factors
The treatment options for calciphylaxis may include:
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There are a number of medications that can be used to treat calciphylaxis, depending on the individual case and the doctor's recommendation.
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It is given intravenously three times a week during dialysis sessions, and acts as a chelating agent to remove calcium from blood vessels and tissues.
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It is taken orally, usually once daily. Cinacalcet lowers the production of PTH and helps control calcium and phosphate levels in the body.
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Penicillin: This is an antibiotic that prevents or treats wound infections caused by bacteria. It is given intravenously or orally, depending on the severity of the infection.
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Anticoagulants: These are blood thinners that prevent or dissolve blood clots in the small blood vessels of the skin. They include apixaban, warfarin, heparin, or low-dose tissue plasminogen activator (TPA). Depending on the type and dose of the medication, it is given intravenously or orally.
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Procedures: Some procedures can be performed to treat calciphylaxis, such as:
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Calciphylaxis can be treated with some procedures, includingroid glands, which produce PTH. This can help lower the levels of calcium and phosphate in the body and prevent further calcium deposits.
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Surgery to remove all or part of the parathyroid glands, which produce PTH, can lower calcium and phosphate levels in the body and prevent further calcium buildups, enzymes, or maggots.
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A hyperbaric oxygen therapy involves breathing pure oxygen in a pressurized chamber, which can increase the amount of oxygen delivered to the affected tissues and improve healing.
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Therapy: Some therapies can be used to treat calciphylaxis, such as:
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Calciphylaxis can be treated with some therapies as hydrocolloid or hydrogel, that keep the wound moist and protect it from infection. The dressings need to be changed regularly and the wound needs to be cleaned and monitored for signs of healing or complications.
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As part of wound care, special dressings are applied to keep the wound moist and protect it from infection. These dressings need to be changed regularly, and the wound should be cleaned and monitored for signs of healinge.
Prevention and Outlook
The prevention of calciphylaxis depends on identifying and addressing the risk factors and the underlying causes of the condition. Some of the preventive measures that can be taken are:
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Following a kidney-friendly diet that is low in phosphorus, calcium, and sodium, and high in protein and fiber
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Taking the prescribed medications and supplements for kidney disease, such as phosphate binders, vitamin D, or calcimimetics, as directed by the doctor
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Avoiding or limiting the use of medications that can trigger or worsen calciphylaxis, such as warfarin, corticosteroids, iron, or calcium supplements
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Maintaining a healthy weight and exercising regularly
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Controlling blood sugar levels and blood pressure
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Quitting smoking and limiting alcohol intake
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Having regular check-ups and blood tests to monitor kidney function and mineral and hormone levels
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Seeking medical attention promptly if any symptoms of calciphylaxis occur
Calciphylaxis has a high mortality rate and a low recovery rate, so its outlook is poor. The survival rate for people with calciphylaxis is estimated to be around 20% to 35% at one year and 10% to 15% at five years. Sepsis, organ failure, and cardiovascular complications are the main causes of death.
However, some factors that can improve the prognosis of calciphylaxis are:
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Early diagnosis and treatment
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Aggressive wound care and infection control
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Effective pain management
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Adequate dialysis and mineral and hormone balance
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Parathyroidectomy or hyperbaric oxygen therapy, if indicated
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Supportive care and palliative care, if needed
It is an uncommon and serious condition that requires urgent medical attention and treatment. By following the preventive measures and the treatment plan, complications and death can be avoided and the quality of life can be improved.
