Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. The inflammation 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.
The cause of GPA is unknown. It can affect people at any age. Men and women are equally affected. It is more common in whites. Symptoms may include joint pain, weakness, tiredness, and cold symptoms such as a runny nose that doesn't get better. Doctors use blood tests, chest X-rays, and biopsies to diagnose GPA and rule out other causes of the symptoms.
Early treatment is important. Most people improve with medicines to slow or stop the inflammation.
NIH: National Institute of Allergy and Infectious Diseases
- ANCA / MPO / PR3 Antibodies Test (American Association for Clinical Chemistry)
- Cyclophosphamide (Cytoxan) (Johns Hopkins Vasculitis Center)
- ESR (Erythrocyte Sedimentation Rate) Test (American Association for Clinical Chemistry)
- Genetics Home Reference: Granulomatosis with polyangiitis (National Library of Medicine)
- Granulomatosis with Polyangiitis (Mayo Foundation for Medical Education and Research)
- Granulomatosis with Polyangiitis (GPA) (National Institute of Allergy and Infectious Diseases)
- Granulomatosis with Polyangiitis (GPA): Know the Signs (Mayo Foundation for Medical Education and Research)
- Granulomatosis with Polyangiitis (Wegener's) (American College of Rheumatology)
- Treating GPA and MPA: What Are the Options? (Mayo Foundation for Medical Education and Research)
- Wegener's Granulomatosis (Johns Hopkins Vasculitis Center)
- ClinicalTrials.gov: Granulomatosis with Polyangiitis (National Institutes of Health)
Journal ArticlesReferences and abstracts from MEDLINE/PubMed (National Library of Medicine)
- Article: Complete heart block caused by diffuse pseudotumoral cardiac involvement in...
- Article: Granulomatosis With Polyangiitis Presenting With an Intracardiac Mass and Complete...
- Article: Tracheobronchial Stenoses in Granulomatosis With Polyangiitis (Wegener's): A Report on...
- Granulomatosis with Polyangiitis -- see more articles