What is Calciphylaxis?
Calciphylaxis is a rare but severe condition mostly seen in patients suffering from advanced kidney disease. However, it can occur in patients with sudden kidney failure, normal kidney function, or earlier stages of chronic kidney disease, which is also known as non-uremic calciphylaxis. This condition, also referred to as calcific uremic arteriolopathy, is characterized by painful skin sores caused by the buildup of calcium in small blood vessels of the skin, leading to tissue damage and death.
Calciphylaxis can cause significant health problems because it results in severe pain, wounds that don’t heal, and frequent hospital visits. It’s a life-threatening condition, with more than 50% of patients dying within a year, usually due to a severe infection known as sepsis.
What Causes Calciphylaxis?
Calciphylaxis is a disease linked with several risk factors. These risk factors can be divided into a few categories: demographics, existing health issues, certain medications, specific kidney disease-related conditions, and a state of increased blood clotting.
Your background or lifestyle can affect your risk. Those of Caucasian ethnicity and women are more likely to suffer from calciphylaxis.
If you have certain health problems, you’re at higher risk as well. These conditions include kidney disease, obesity, diabetes, hypoalbuminemia (low levels of a protein called albumin in the blood), autoimmune disorders like lupus and rheumatoid arthritis, liver disease, cancer, and a history of regular dialysis treatment.
Several medications can also increase your likelihood of getting calciphylaxis. These include warfarin, a blood thinner; corticosteroids, which reduce inflammation and suppress the immune system; calcium-based drugs that bind to phosphate; hormone treatments related to vitamin D; and iron therapy.
Besides, there are specific conditions related to kidney disease that can also be risk factors. These include hyperphosphatemia (high phosphate levels in your blood), hypercalcemia (high calcium levels in your blood), hyperparathyroidism (overactivity of the parathyroid glands), and adynamic bone disease, a type of bone disorder.
Lastly, if you have a state where your blood clots more easily than normal or have had tissue damage from injections like insulin, your risk of calciphylaxis rises.
Risk Factors and Frequency for Calciphylaxis
Calciphylaxis is a condition that can occur even if you don’t have kidney disease, but it’s most commonly found in patients with severe kidney failure. The occurrence of calciphylaxis in those undergoing dialysis varies between 0.04% and 4%, and this rate has been steadily increasing over the past ten years.
Signs and Symptoms of Calciphylaxis
Calciphylaxis is a condition that mainly affects the skin, causing a lot of pain. It often starts as painful skin lesions or painful lumps under the skin. Sometimes, the pain shows up before any visible signs on the skin do. These lumps or skin lesions can be reddish-purple or red bumps or flat areas on the skin. Changes like a lace-like pattern on the skin can also appear due to poor blood supply. As the condition worsens, these skin changes may become open sores with tissue death, hardened skin, and infection. Areas of the body with more fatty tissue, such as the stomach, thighs, and buttocks, are most often affected by this condition, but it can also affect areas like fingers and toes. Extreme pain is a major concern with calciphylaxis, causing significant discomfort.
Even though it impacts the skin mostly, calciphylaxis is a condition that can affect the whole body, including organs like the eyes, penis, muscles, brain, intestines, and lungs.
Testing for Calciphylaxis
Diagnosing a condition called calciphylaxis can be quite tricky and requires doctors to be very alert. The definite way to diagnose it is by taking a small sample, known as a biopsy, from the affected areas of the skin. This sample is then tested, revealing different things like calcification of dermal arterioles or changes in small arteries, and signs of tissue death or narrowing of blood vessels.
However, some doubts exist about whether or not a biopsy is always needed for diagnosis. This is because the biopsy procedure itself carries some risks, especially for patients who just have small lumps beneath the skin. The process of obtaining the sample can sometimes cause complications like open sores, bleeding, infection, or even tissue death. Therefore, in situations where the doctor is reasonably confident about the presence of calciphylaxis, they may skip the biopsy and move straight to treatment.
In addition to the biopsy, doctors also carry out a range of blood tests, including checking kidney and liver function, clotting time, the balance between plasma and red cells, and protein levels. Sometimes, they might also check for a tendency to abnormal clot formation.
There are also a few other methods that are still experimental but can help diagnose calciphylaxis. One of these is using a bone scan to check for tissue calcification in the areas where the lesions are found. Another approach is checking the levels of a protein called fetuin-A in the bloodstream, which may be helpful in evaluating for calciphylaxis.
Treatment Options for Calciphylaxis
Calciphylaxis is a condition that might be caused by disorders affecting the bone and minerals in our body. To manage this, it’s important to prevent high levels of calcium in the blood and keep phosphorus levels under 5.5 mg/dL. Dialysis, a treatment to clean the blood when kidneys can’t, should be done effectively and more often if needed to better remove harmful substances from the blood and improve markers related to bone and mineral health.
Patients should stop taking calcium supplements and instead use medications that don’t contain calcium to lower phosphate levels in the blood. They should also avoid using dialysis solutions that have high levels of calcium (more than 2.5 meq/L). It’s necessary to keep the parathyroid hormone, which controls calcium levels in the body, between 150-300 ng/mL.
If patients have a condition called secondary hyperparathyroidism, they should use a drug called cinacalcet instead of activated vitamin D, as the drug helps to lower parathyroid hormone without increasing calcium and phosphorus levels in the blood. A study found that those who took cinacalcet had a lower chance of getting calciphylaxis. Another drug that acts similarly, etelcalcetide, hasn’t been studied for calciphylaxis yet, but may be suitable for patients who can’t tolerate cinacalcet.
In addition to controlling bone and mineral levels, patients should stay away from drugs that can make calciphylaxis worse. For example, warfarin may increase the risk of calciphylaxis, so patients should know the benefits and risks of stopping the drug or switching to a different blood thinner. Studies are looking into using vitamin K to treat calciphylaxis, but it’s not yet known if this is effective.
Sodium thiosulfate is often used as an unofficial treatment for calciphylaxis, and its effect on the condition is promising, though the exact way in which it works isn’t fully understood. Some potential side effects of this drug include metabolic acidosis and fluid overload.
Proper wound care and pain management are also important in managing calciphylaxis. Dead and damaged tissue may need to be surgically removed to prevent infection and encourage healing. Although preventive use of antibiotics isn’t suggested, they might be needed if an infection is likely. Some reports have also suggested that oxygen therapy can help heal wounds in some cases.
Lastly, there have been cases in which calciphylaxis improved or even completely disappeared in patients with severe kidney disease after a kidney transplant. However, the condition can also occur after a transplant, and the immunosuppressant drugs used during the transplant process could increase the risk for skin infections.
What else can Calciphylaxis be?
If a person is suffering from calciphylaxis, which is a serious condition, there could be other diseases or conditions which present similar symptoms. These include:
- Skin necrosis, associated with Warfarin, a blood-thinning medication
- Antiphospholipid syndrome, which causes blood to clot more easily than normal
- Cholesterol embolization, where cholesterol crystals block blood vessels
- Vasculitis, an inflammation of blood vessels
- Cellulitis, a common infection of the skin and tissues beneath it
In order to distinguish calciphylaxis from these other possible conditions, doctors will take into account the patient’s medical history, perform a physical examination, run laboratory tests, and assess tissue under a microscope.
What to expect with Calciphylaxis
Calciphylaxis carries a high risk, with mortality rates between 45% and 80% within the first year. This condition also does not respond well to treatments. Particularly, patients with ulcerated sores are highly likely to develop infections, which is the main cause of death.
Possible Complications When Diagnosed with Calciphylaxis
The main complication of this disease is additional infections in the impacted areas. Patients with this disease often have weakened immune systems, which greatly increases the risk of sepsis, a life-threatening infection that can spread throughout the body.
A less severe but still extremely troublesome issue associated with this disease is debilitating pain. Lesions caused by this disease, known as calciphylaxis, are extremely painful and can greatly reduce a patient’s quality of life.
Common complications related to this disease include:
- Additional infections in the affected areas
- High risk of sepsis due to a weakened immune system
- Severe pain from calciphylaxis lesions
- Reduced quality of life
Preventing Calciphylaxis
Preventing calciphylaxis, a serious health condition, is extremely important. Patients should be advised to stick to their hemodialysis treatment schedule, follow dietary restrictions, and use phosphate binders. Following these guidelines can help prevent the development of this condition.