Churg-Strauss syndrome

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Tests and diagnosis

By Mayo Clinic staff

There are no specific tests to confirm Churg-Strauss syndrome, and signs and symptoms are similar to those of other diseases, so it can be difficult to diagnose. To help make diagnosis easier, the American College of Rheumatology has established criteria for identifying Churg-Strauss syndrome.

The six criteria
The disease is generally considered to be present if a person has four of the six criteria, but your doctor may feel confident diagnosing Churg-Strauss even if you meet only two or three of the criteria, which include:

  • Asthma
  • Eosinophilia — eosinophils normally make up 1 to 3 percent of white blood cells; a count higher than 10 percent is considered abnormally high and a strong indicator of Churg-Strauss syndrome
  • Mononeuropathy or polyneuropathy — damage to one or more nerve groups
  • Pulmonary infiltrates — spots or lesions on a chest X-ray that move from one place to another or come and go
  • History of acute or chronic sinus pain
  • Extravascular eosinophils — white cells that are present outside your blood vessels

To help determine whether you meet any of these criteria, your doctor is likely to request several tests, including:

  • Blood tests. When your immune system attacks your body's own cells, as happens in Churg-Strauss syndrome, it forms proteins called autoantibodies. A blood test can detect certain autoantibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. A blood test also can measure the level of eosinophils, although an increased number of these cells may be caused by other diseases, including asthma.
  • Imaging tests. X-rays, computerized tomography (CT) scans and magnetic resonance imaging (MRI) look for abnormalities in your lungs and sinuses.
  • Biopsy of affected tissue. If other tests suggest Churg-Strauss syndrome, you may have a small sample of tissue (biopsy) removed for examination under a microscope. The doctor may remove tissue from your lungs or another organ, such as skin or muscle, to confirm or rule out the presence of vasculitis. A biopsy is usually performed only on a site that has shown some abnormality on another test.
References
  1. What is Churg Strauss syndrome? Churg Strauss Syndrome Association. http://www.cssassociation.org/about_the_syndrome.asp. Accessed Aug. 20, 2008.
  2. King TE. Churg-Strauss syndrome (allergic granulomatosis and angiitis). http://www.uptodate.com/home/index. Accessed Aug. 20, 2008.
  3. Churg Strauss syndrome. Vasculitis Foundation. http://www.vasculitisfoundation.org/churgstrausssyndrome. Accessed Aug. 20, 2008.
  4. Hellmich B, et al. Update on the pathogenesis of Churg-Strauss syndrome. Clinical and Experimental Rheumatology. 2003;21(6)(suppl):S69-S77.
  5. Lilly CM, et al. Asthma therapies and Churg-Strauss syndrome. The Journal of Allergy and Clinical Immunology. 2002;109(1):S1-S19.
  6. Weschler ME, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. 2000;117(3):708-713.
  7. Hauser T, et al. The leukotriene receptor antagonist montelukast and the risk of Churg-Strauss syndrome: A case-crossover study. Thorax. 2008;63:677-682.
  8. Tsurikisawa N, et al. Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin. Annals of Allergy, Asthma & Immunology. 2004;92(1):80-87.

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Nov. 14, 2008

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