Churg-Strauss Disease (also known as EGPA) is an inflammation of small and medium-sized blood vessels that can damage your nose, sinuses, lungs, heart, kidneys, and nerves. It can also cause asthma, fever, and skin problems.
Medications called steroids — such as prednisone — can help control symptoms. These drugs stop your body’s immune system, which normally fights germs, from attacking your organs and tissues.
Symptoms
The symptoms of Churg-Strauss syndrome are caused by inflammation in the blood vessels that restricts blood flow to organs and tissues. This condition can affect all parts of the body, but it mostly causes problems in the lungs. Symptoms can include wheezing, difficulty breathing, cough, fever, and fatigue. Other symptoms can be more serious and include skin problems, heart disease, kidney failure, and nerve damage. Churg-Strauss syndrome can be fatal if left untreated.
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Doctors diagnose the disorder by taking a sample of your blood and checking it for signs of inflammation or damage to your blood vessels. They may also take X-rays or CT scans to look for damage to the airways in your lungs, or for growths called polyps inside your nose. A sample of tissue from your lungs or other parts of the body is also checked in a laboratory to confirm the diagnosis. Your doctor may also do a test to measure your lung function or a blood test called a CBC to check your general health and to detect some of the complications that can develop with this condition.
People with EGPA often have allergies, and asthma is a common symptom. Asthma symptoms may start a year or more before the more generalized systemic symptoms of Churg-Strauss Syndrome appear. Some experts believe that certain medications used to treat asthma, such as leukotriene receptor antagonists or montelukast, can precipitate the onset of this condition.
In EGPA, abnormal clusters of eosinophils, a type of white blood cell that usually fights parasite infections, cause inflammation of small and medium-sized blood vessels. This restricts blood flow and can lead to a formation of granulomas in the affected areas. Eosinophils are also found in high levels in the blood and tissues of people with this condition, which helps doctors distinguish it from other inflammatory disorders such as Wegener’s granulomatosis, polyarteritis nodosa, or Guillain-Barre syndrome.
People of all ages and both genders can develop EGPA, but the disease is most common in people between the ages of 38 and 54. It is not known what causes the condition, but it can run in families.
Diagnosis
Churg-Strauss Syndrome is hard to diagnose because it often doesn’t have clear symptoms. Other diseases can cause the same symptoms, so it’s important for your doctor to rule out these other conditions with a physical exam and a careful history. You’ll also have blood and imaging tests, including X-rays of the lungs and sinuses. These tests can help your doctor see inflammation in your small and medium-sized blood vessels, which is a key feature of this disease. They can also find granulomas, or clumps of white blood cells, that form in blood vessels when the inflammation causes them to swell.
The inflammation in Churg-Strauss Syndrome can affect the lungs, sinuses, kidneys, and nerves. It also can cause heart problems, such as swelling of the blood vessels in your lungs (vasculitis). If it gets to your kidneys, you may develop a type of kidney damage called glomerulonephritis. This damage can lead to kidney failure. It can also affect your nerves, causing numbness or pain in your arms and legs.
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In this condition, your body makes high levels of a type of white blood cell called eosinophils. These cells normally help fight infections. But in people with Churg-Strauss Syndrome, eosinophils build up to the point where they make up 10% or more of the white blood cells in your body. Your doctor will check your blood for these cells and take a sample of tissue from the affected area, such as your nose or skin, to look for them under a microscope.
If you have the disease, your doctor will prescribe medication to treat it. Most doctors start with a drug called prednisone, which reduces inflammation and can treat many of the problems caused by this disease. Doctors will slowly decrease your dose until you are on the lowest dose that controls your symptoms.
You’ll need to visit your doctor frequently to monitor the effects of your medication. Some people can have serious side effects, such as a lack of energy or weight gain from steroids. Other side effects include bone loss, increased risk of infection and diabetes, high blood pressure, and cataracts.
Treatment
Churg-Strauss syndrome, also called eosinophilic granulomatosis with polyangiitis (EGPA), is a rare condition that causes inflammation of blood vessels (vasculitis). Symptoms vary depending on which organs are affected. It can affect the lungs, heart, kidneys, brain, and nervous system. It can be fatal if not treated. EGPA is a form of vasculitis caused by a buildup of white blood cells that attack small blood vessels. It can cause other disorders, including rashes, kidney disease, and loss of feeling in the hands and feet. Symptoms of the disorder can vary between people and even change over time.
About two out of three people with Churg-Strauss syndrome have a rash, often on the elbows. The rash can be red, purple, or scaly. It can leave bumps that heal into scars. The rash can cause pain or itching. Inflammation of the skin can damage nerves that deliver feelings to the fingers and toes. People can have numbness, burning, or tingling sensations in the fingers and toes. EGPA can also lead to kidney problems, such as a decrease in how much urine you produce or a loss of protein in the urine (proteinuria).
Almost everyone with Churg-Strauss syndrome has asthma — swelling of the airways in the lungs. It can make it difficult to breathe, especially when you have a cold or the flu. In addition, it can cause nasal congestion, stuffiness, and thick discharge from the nose. Some people with this condition have polyps in their sinuses, which can block the nose and throat. Others have gastrointestinal problems, such as colitis or ulcers in the digestive tract.
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Blood tests can help confirm the diagnosis. These tests check for high levels of a type of white blood cell called eosinophils, which are part of the immune system’s defense against germs. They can also test for a substance called antineutrophil cytoplasmic antibodies, or ANCA, which is made when the immune system attacks its own tissues. The ANCA test is used to help diagnose Wegener’s granulomatosis and other similar conditions, but it can also be found in some people with Churg-Strauss.
