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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Granulomatosis with polyangiitis (Wegener's)


Other Names for this Disease

  • Midline granulomatosis
  • Wegener granulomatosis
  • WG
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Tests & Diagnosis

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How is Wegener's granulomatosis diagnosed?

Wegener's granulomatosis (WG) has a wide variety of ways that it may present itself. Affected individuals may first report some chronic, nonspecific complaints including fevers, night sweats, fatigue, lethargy, loss of appetite and weight loss; this may make the diagnosis difficult at first.[1] However, to treat people with WG most effectively, healthcare professionals must diagnose the disease early.[2]

There are no specific blood tests that can be used to diagnose WG, but blood tests are important to rule out other causes of illness and to determine which organ may be affected. Most blood tests can only suggest that a person has inflammation somewhere in the body. Anemia (low red blood cell count), elevated white blood cell count and platelet count, and an elevated sedimentation rate are commonly found in people with Wegener's granulomatosis. If the kidneys are involved, a healthcare professional can see red blood cells and structures called red blood cell casts in the urine when viewed under a microscope, and the blood tests measuring kidney function may show abnormalities. X-ray results can also be very helpful in diagnosing WG. People with lung problems will have abnormal chest X-rays. CT (computed tomography) scans in people with sinus problems may show thickening of the sinus lining. Many people with active WG have a blood test that shows the presence of a specific type of antibody, a disease-fighting protein, called antineutrophil cytoplasmic antibodies (ANCA). Although a positive ANCA test is useful to support a suspected diagnosis of WG, in most instances healthcare professionals do not use it by itself to diagnose this disorder. Furthermore, The ANCA test may be negative in some people with active Wegener's granulomatosis.[2]

Currently, the only clear-cut way to diagnose Wegener's granulomatosis is by performing a biopsy (removing a tiny piece of tissue) of an involved organ (usually the sinuses, lung, or kidney). A healthcare professional will examine tissue from the organ under the microscope to confirm the presence of vasculitis and granulomas (a specific type of inflammation), which together are features of Wegener's granulomatosis. A biopsy is very important both to confirm the presence of the disorder and also to make sure other disorders that may have similar signs and symptoms are not present.[2]
Last updated: 7/27/2011

References
  1. Patricia J Papadopoulos. Wegener Granulomatosis. eMedicine. April 20, 2011; http://emedicine.medscape.com/article/332622-overview. Accessed 7/27/2011.
  2. Wegener's Granulomatosis. NIAID. April 26, 2011; http://www.niaid.nih.gov/topics/wegeners/Pages/Default.aspx. Accessed 7/27/2011.


Other Names for this Disease
  • Midline granulomatosis
  • Wegener granulomatosis
  • WG
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.