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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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Your Question

My husband has been diagnosed with Wegener's granulomatosis. How might this condition be treated? How can I learn about research involving this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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 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][3] 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

How can I learn about research involving Wegener's granulomatosis?

Research scientists at the National Institutes of Allergy and Infectious Diseases (NIAID) have been studying Wegener's granulomatosis since the 1970s. While strides have been made towards treating this condition, the side effects experienced by many patients remains a concern. That is why researchers continue to study this disease, in the hopes of better understanding its causes and to develop new and better treatments.[4]

NIAID and other parts of the National Institutes of Health (NIH) support research on Wegener's granulomatosis and related forms of vasculitis at medical centers throughout the United States through the extramural grants program. NIH supports the Vasculitis Clinical Research Consortium (VCRC), for example. The multicenter VCRC fosters and facilitates clinical investigation in the inflammatory vasculitides, including Wegener's granulomatosis.[4]

VCRC has four major U.S. vasculitis centers:

  • Boston University School of Medicine, Massachusetts
  • The Cleveland Clinic, Ohio
  • The Johns Hopkins Vasculitis Center, Baltimore, Maryland
  • The Mayo Clinic College of Medicine, Rochester, Minnesota

More information about VCRC is available at: http://rarediseasesnetwork.epi.usf.edu/vcrc/. Much of the information available at this site is designed to help people manage their disease.

The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. ClinicalTrials.gov lists trials that are studying or have studied Wegener's granulomatosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies. After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if you are eligible for any clinical trials. If you are located outside the United States, and would like to be contacted via telephone, you will need to provide your telephone number in full, including area code and international dialing prefix.

Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Email: prpl@mail.cc.nih.gov
Web site:  http://clinicalcenter.nih.gov/

If you are interested in enrolling in a clinical trial, you can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.
http://clinicaltrials.gov/ct2/info/understand

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases Research (ORDR), part of the National Institutes of Health.
http://rarediseases.info.nih.gov/Resources.aspx?PageID=8 

The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, click on the link and enter the disease name in the "Terms Search" box. Then click "Submit Query".
Link: http://projectreporter.nih.gov/reporter.cfm
Last updated: 6/8/2011

References