Summary
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.
Symptoms
- Granulomatosis with Polyangiitis (GPA): Symptoms and Causes (Mayo Foundation for Medical Education and Research)
Diagnosis and Tests
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Antineutrophil Cytoplasmic Antibodies (ANCA) Test
(National Library of Medicine) Also in Spanish
-
Erythrocyte Sedimentation Rate (ESR)
(National Library of Medicine) Also in Spanish
Treatments and Therapies
- Cyclophosphamide (Cytoxan) (Johns Hopkins Vasculitis Center)
Genetics
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Granulomatosis with polyangiitis: MedlinePlus Genetics
(National Library of Medicine)
Clinical Trials
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ClinicalTrials.gov: Granulomatosis with Polyangiitis
(National Institutes of Health)
Journal Articles References and abstracts from MEDLINE/PubMed (National Library of Medicine)
- Article: Vasculitis Activity-Predicting Ability of IL-12 Family Cytokines in Patients with Microscopic...
- Article: Activity of granulomatosis with polyangiitis and its correlation with mTOR phosphoproteomics...
- Article: The complex HLA-E-nonapeptide in Behçet disease.
- Granulomatosis with Polyangiitis -- see more articles
Reference Desk
- Vasculitis Terms A to Z (Vasculitis Foundation)
Patient Handouts
- Granulomatosis with polyangiitis (Medical Encyclopedia) Also in Spanish