Granulomatosis with Polyangiitis
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.
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The immune system begins to attack healthy blood vessels and causes inflammation. It is not clear what causes this change in the immune system.
GPA is more common in people over 65 years of age.
Symptoms will vary. They can range from mild to serious. Ear, nose, and throat symptoms often appear first. They are common but do not respond to normal care. GPA can cause:
- Achy joints and muscles
- Overall feeling of discomfort or tiredness
- Lack of hunger
- Weight loss
A person with GPA can also have:
- Hearing problems
- Pain in the ear or the middle part of the face
- Lasting nasal discharge, with crusts or sores that do not heal
- Frequent nosebleeds
- Ear discharge and frequent ear infections
- Ulcers in the mouth and nose
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The doctor will ask about symptoms and health history. Tests may be needed to rule out other conditions. The doctor may suspect GPA based on symptoms. A blood test will help confirm it. A sample of the tissue may also be taken for more tests. Other tests may be done to look for any changes in kidneys or lungs.
The goal of treatment is to reduce swelling. Medicines can be given to:
- Ease swelling in the arteries
- Decrease the reaction of the immune system
The choice of medicine will depend on what organs are affected. Treatment may last several months or years.
GPA cannot be prevented.
- Berden, A., Göçeroglu, A., et al. Diagnosis and management of ANCA associated vasculitis. BMJ, 2012; 344: e26
- Granulomatosis with polyangiitis. EBSCO DynaMed website. Available at: http://www.dynamed.com/condition/granulomatosis-with-polyangiitis-10.
- 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.
- Yates, M., Watts, R.A., 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; 75 (9): 1583.
- Michael J. Fucci, DO, FACC
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