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
- Granulomatosis with Polyangiitis (GPA): Symptoms and Causes (Mayo Foundation for Medical Education and Research)
Treatments and Therapies
- Cyclophosphamide (Cytoxan) (Johns Hopkins Vasculitis Center)
- Genetics Home Reference: granulomatosis with polyangiitis (National Library of Medicine)
- ClinicalTrials.gov: Granulomatosis with Polyangiitis (National Institutes of Health)
Journal Articles References and abstracts from MEDLINE/PubMed (National Library of Medicine)
- Article: Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal...
- Article: Pulmonary Vasculitis: Spectrum of Imaging Appearances.
- Article: Antineutrophil cytoplasmic antibody positivity in IgG4-related disease: A case report...
- Granulomatosis with Polyangiitis -- see more articles