Wegener's granulomatosis


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Definition

By Mayo Clinic staff

Wegener's granulomatosis (VEG-eh-nerz gran-u-loe-muh-TOE-sis) is an uncommon disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs.

Wegener's granulomatosis, which is also called granulomatosis with polyangiitis (GPA), often affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious.

Wegener's granulomatosis also produces a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue. There is no known cause for Wegener's granulomatosis.

Early diagnosis and treatment of Wegener's granulomatosis may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal, most commonly from kidney failure.

References
  1. Longo DL, et al. Harrison's Online. 18th ed. New York, N.Y.: The McGraw-Hill Companies; 2012. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=4. Accessed Oct. 12, 2012.
  2. Granulomatosis with polyangiitis (Wegener's). American College of Rheumatology. http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/wegeners.asp. Accessed Oct. 12, 2012.
  3. Holle JU, et al. Clinical manifestations and treatment of Wegener's granulomatosis. Rheumatologic Disease Clinics of North America. 2010;36:507.
  4. Papadakis MA, et al. Current Medical Diagnosis & Treatment 2013. 52nd ed. New York, N.Y.: The McGraw-Hill Companies; 2013. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=1. Accessed Oct. 12, 2012.
  5. Falk RJ, et al. Clinical manifestations and diagnosis of Wegener's granulomatosis and microscopic polyangiitis. http://www.uptodate.com/index. Accessed Sept. 21, 2012.
  6. FDA approves Rituxan to treat two rare disorders. U.S. Food and Drug Administration. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm251946.htm. Accessed Aug. 22, 2012.
  7. Langford CA. Update on the treatment of granulomatosis with polyangiitis (Wegener's). Current Treatment Options in Cardiovascular Medicine. 2012;14:164.
  8. Turnball J, et al. Adverse effects of therapy for ANCA-associated vasculitis. Best Practice and Research Clinical Rheumatology. 2009;23:391.
DS00833 Dec. 19, 2012

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