Granulomatosis with polyangiitis (GPA) is a rare disease of the blood vessels. The walls of blood vessels become inflamed. This slows the flow of blood to tissue. It can lead to damage of connected organs. Left untreated it can be fatal.
The immune system begins to attack healthy blood vessels. This causes inflammation. It’s not clear what causes this change in the immune system.
GPA is more common in people over 65 years old.
Symptoms will vary. They can range from mild to serious. Ear, nose, and throat symptoms often appear first. They are common but don’t respond to normal care. GPA can cause:
Other common problems:
The doctor will ask about your symptoms and past health. Tests may be needed to rule out other conditions. The doctor may suspect GPA based on your symptoms. A blood test will help to confirm it. A sample of the tissue may also be taken. It will be looked at in a lab for more details. Other tests may be done to look for any changes in kidneys or lungs.
Treatment can help to reduce inflammation. It can create a period of time without disease called remission.
The choice of medicine will depend on what organs are affected. Treatment may last several months or years.
There is no way to prevent GPA since the cause is unknown.
National Institute of Allergy and Infectious Diseases (NIH)
College of Family Physicians of Canada
Berden A, Göçeroglu A, Jayne D, et al. Diagnosis and management of ANCA associated vasculitis. BMJ. 2012 Jan 16;344:e26
Granulomatosis with polyangiitis. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T115969/Granulomatosis-with-polyangiitis. Updated March 5, 2020. Accessed March 18, 2020.
Granulomatosis with polyangiitis. National Institute of Allergy and Infectious Diseases website. Available at: https://www.niaid.nih.gov/topics/gpa/pages/default.aspx. Updated October 29, 2013. Accessed March 18, 2020.
Granulomatosis with polyangiitis (GPA). Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/granulomatosis-with-polyangiitis-gpa. Updated September 2017. Accessed March 18, 2020.
Yates M, Watts RA, Bajema IM, et al. European League Against Rheumatism/European Renal Association/European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016 Sep;75(9):1583.
Last reviewed March 2020 by EBSCO Medical Review Board Michael J. Fucci, DO, FACC Last Updated: 3/18/2020