Churg Strauss vasculitis (CSV) is an extremely rare disease that results from inflammation of the blood vessels and causes injury to many organ systems. Vasculitis means inflammation of blood vessels and CSV may damage small and medium-sized blood vessels. CSV also is known as Churg Strauss syndrome, allergic angiitis, and granulomatosis.
The areas most commonly involved are the nose, sinuses, lungs, heart, intestines, and nerves. In about one-half to two-thirds of people with CSV, inflammation also may affect the kidneys, muscles, or joints.
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CSV is distinguished from other types of vasculitis because people with CSV often have asthma and/or have an increased number of so-called allergic type blood cells called "eosinophils."
What Are the Symptoms of Churg Strauss Vasculitis?
Because many different organ systems may be involved, many different symptoms are possible. People who have CSV may feel: generally ill, have fevers, shortness of breath, cough, wheezing, increased nasal discharge, facial pain from sinusitis, rashes, abdominal pain, blood in the stools, or muscle and joint pain (arthritis).
CSV in the nerves may cause an abnormal sensation called paresthesia, followed by loss of sensation or loss of strength. Any combination of these symptoms may be present.
Who Gets Churg Strauss Vasculitis?
CSV can affect people of all ages, from children to the elderly. The average age of people with CSV at diagnosis is about 35 to 45 years old.
What Causes Churg Strauss Vasculitis?
Although allergies have been thought to play a role in the development of CSV in some people, the exact cause is unknown.
How Is Churg Strauss Vasculitis Diagnosed?
The diagnosis of CSV is based on a combination of factors, including:
Complete medical history and physical exam to exclude other illnesses that may have similar symptoms
Presence of typical CSV symptoms, especially the presence of asthma, the most constant feature of CSV.
Blood tests that indicate abnormal blood counts, often with an increased number of eosinophils
X-rays that show tissue damage or inflammation in areas such as the lungs or sinuses
Urinalysis, which may indicate excessive protein or an abnormally high number of red blood cells
Once the diagnosis of CSV is suspected, a biopsy (tissue sample) of an affected area can confirm that inflammation of blood vessels is present. This is most easily done if there is a suspicious rash.
If easily accessible areas, such as the skin, are not affected, the diagnosis may be supported by abnormal findings on a kidney or lung biopsy. These organs would be recommended for biopsy only if there were abnormal findings during an exam or on X-rays. If the results of blood or urine tests imply kidney involvement, a biopsy to show how the kidney is affected may be useful.