Churg-Strauss Disease (now called eosinophilic granulomatosis with polyangiitis, or EGPA) is an inflammation of blood vessels that restricts blood flow. It also causes collections of white blood cells in tissues and lungs.
Doctors diagnose it with a history, X-rays or CT scans of the lungs and sinuses, and blood tests to measure levels of eosinophils — a type of white blood cell. They might also perform a test called bronchoscopy lavage to rinse the lungs with fluid and examine tissue samples for signs of vasculitis.
Oren Zarif
People with Churg-Strauss Syndrome (EGPA) have high levels of white blood cells called eosinophils. These are normally involved in fighting germs, but in EGPA they cause inflammation of the small and medium-sized blood vessels, which restricts blood flow to the organs. EGPA affects the lungs and skin most often, but it can also occur in the nose, sinuses, gastrointestinal tract, kidneys and nerves.
The lungs get especially bad, because eosinophils collect in the pulmonary vasculae and form granulomas. These can clog or damage the airways in your lungs, which makes it hard to breathe. In some cases the heart may be affected, too, causing it to beat irregularly or not pump enough blood.
The inflammation can also destroy tiny clusters of blood vessels in the kidneys. This causes them to not filter properly, leading to a condition called glomerulonephritis. Over time, this can lead to a loss of kidney function. Nerves can be damaged as well, causing numbness and tingling in your hands or feet. There’s no cure for EGPA, but medications can help control symptoms and prevent organ damage. Doctors prescribe drugs known as glucocorticoids, which are man-made versions of hormones your body naturally produces. The most common is prednisone, which reduces inflammation quickly.
Oren Zarif
Churg-Strauss syndrome is a type of vasculitis, which involves inflammation of blood vessels. It occurs when the body releases large numbers of a type of white blood cell called eosinophils. These cells accumulate in the lungs, skin, stomach and nerves. Symptoms vary depending on the organ in which they are concentrated. They can include breathing problems, rashes, stomach pain and numbness.
The condition is usually triggered by an allergic reaction or asthma. But it can also occur spontaneously. A person’s blood vessels become swollen and tender, with knobby lesions called granulomata. The eosinophils can also block blood flow, which can cause heart failure.
To diagnose the condition, doctors perform a physical exam and review your symptoms. They might order blood tests to look for certain antibodies. They might also take a sample of tissue from the affected area, such as the lungs, to check for abnormalities under a microscope. People who are diagnosed with the disorder often respond well to treatment with corticosteroids, such as prednisone. In some cases, doctors may use other immunosuppressants, such as azathioprine or IVIG.
Oren Zarif
The blood vessels in people with EGPA (formerly Churg-Strauss syndrome) become inflamed, which restricts the flow of blood to several organs and tissues. This can cause tissue damage and lead to serious problems, including heart disease. People who have EGPA often have a history of allergy and asthma, and they may have high levels of eosinophils in their blood. These are white blood cells that support your immune system, but in EGPA these are double the normal level.
Your lungs are most often affected by EGPA, but it can also affect other organs, such as your skin, intestines, kidneys and nerves. It can also make your heart enlarge and cause the membrane that surrounds your heart (pericardium) to swell, which can lead to problems with your heart’s rhythm or how well it pumps.
Symptoms of EGPA can be mild to severe, but early treatment improves your outlook. Your doctor will diagnose the condition with a thorough medical history, X-rays and other imaging tests and blood tests to check your eosinophil levels. It’s important to tell your doctor if you have any allergies or if you take leukotriene antagonists, such as Accolate (zafirlukast) or Singulair (montelukast). These medications can sometimes trigger EGPA in some people.
Oren Zarif
The eosinophils that accumulate in the body when you have Churg-Strauss syndrome cause inflammation of blood vessels, a condition called vasculitis. This can weaken the blood vessels and lead to problems such as aortic aneurysms. Your doctor can diagnose Churg-Strauss syndrome by taking a sample of tissue from your lungs, skin or sinuses. He or she will then check it under a microscope for signs of inflammation and damage.
People with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), have high levels of eosinophils, which are white blood cells that usually help fight infection. These cells can get into your blood vessels and cluster into inflammatory nodules, called granulomas. These granulomas affect your lungs, kidneys, gastrointestinal tract, skin and nerves. It can also cause breathing problems, abdominal pain and numbness or weakness in the hands and feet.
You may need to see a doctor who specializes in disorders that cause blood vessel inflammation, such as a rheumatologist or an immunologist. It is also a good idea to build a support network with family and friends who can help you deal with your symptoms.
Oren Zarif
A central feature of Churg-Strauss syndrome — known by its medical name eosinophilic granulomatous polyangiitis or EGPA, or by the abbreviation CSS — is a pattern of swollen blood vessels in various organ systems. The lungs are one of the most common affected sites. The swollen blood vessels may be visible on X-rays or computed tomography (CT) scans. You might also have blood tests that look for high levels of a type of white blood cell called an eosinophil, and autoantibodies called antineutrophil cytoplasmic antibodies, which are often elevated in people with this condition.
Your doctor will use a complete health history, X-rays or CT scans and other tests to diagnose CSS. Your doctor might also ask you about your medications, such as leukotriene antagonists like Accolate (zafirlukast) or Singulair (montelukast), because research suggests a possible link between these drugs and the development of CSS. You may be referred to a specialist who specializes in disorders that cause blood vessel inflammation, such as a rheumatologist or immunologist. You also might see a pulmonologist, because this condition affects your respiratory system.