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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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Overview



What is Wegener's granulomatosis?

How is Wegener's granulomatosis diagnosed?

How might Wegener's granulomatosis be treated?


What is Wegener's granulomatosis?

Wegener's granulomatosis is a type of vasculitis, or inflammation of the blood vessels. This limits the flow of blood to important organs, causing damage. It can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs and kidneys. Symptoms can vary in nature and severity, and may include sinus pain; discolored or bloody fluid from the nose; nasal ulcers; constant runny nose (rhinorrhea); joint pain; weakness; tiredness; and/or skin lesions.[1][2] The cause of Wegener's granulomatosis is unknown. Early treatment is important. Most people improve with medicines to slow or stop the inflammation.[1]
Last updated: 7/27/2011

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.[3] 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

How might Wegener's granulomatosis be treated?

Treatment of Wegener's granulomatosis usually includes a combination of glucocorticoids such as prednisone and an immunosuppressive drug such as cyclophosphamide, methotrexate, or azathioprine.[1][4] These medicines may cause serious side effects. Patients should be carefully monitored by a healthcare provider. Other medications which may be prescribed include:[1]

  • Bisphosphonate (Fosamax) to prevent bone loss caused by prednisone
  • Folic acid or folinic acid (leucovorin) -- if you are taking methotrexate
  • Trimethoprim/sulfamethoxazole to prevent lung infections
More detailed information about the treatment of Wegener's granulomatosis can be accessed through the
National Institute of Allergy and Infectious Diseases (NIAID)

eMedicine also provides information about the treatment of Wegener's granulomatosis, including a section detailing alternative and/or promising therapies.
Last updated: 7/27/2011

References
  1. Wegener's Granulomatosis . MedlinePlus. 2011; http://www.nlm.nih.gov/medlineplus/wegenersgranulomatosis.html. Accessed 4/7/2011.
  2. Wegener's Granulomatosis. NIAID. April 26, 2011; http://www.niaid.nih.gov/topics/wegeners/Pages/Default.aspx. Accessed 7/27/2011.
  3. Patricia J Papadopoulos. Wegener Granulomatosis. eMedicine. April 20, 2011; http://emedicine.medscape.com/article/332622-overview. Accessed 7/27/2011.
  4. Disease Definitions: Wegener's Granulomatosis. Vasculitis Clinical Research Consortium. http://rarediseasesnetwork.epi.usf.edu/vcrc/learnmore/definitions.htm#wg. Accessed 4/7/2011.