Granulomatosis with Polyangiitis (Wegener's Granulomatosis)
What is Granulomatosis with Polyangiitis (Wegener's Granulomatosis)?
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disease that causes inflammation of the blood vessels (vasculitis), and inflammation of smaller blood vessels (polyangiitis), which can restrict blood flow to various organs, and the formation of granulomas (masses of inflammatory tissue). It primarily affects tissues and organs that rely on smaller blood vessels, such as the upper respiratory tract, lungs and kidneys, but can involve other organs in the body as well.
Synonyms
- Wegener’s Granulomatosis (historic term)
- Necrotizing Granulomatous Vasculitis
- ANCA-associated vasculitis (AAV), specifically PR3-ANCA associated vasculitis
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disease that causes inflammation of the blood vessels (vasculitis), and inflammation of smaller blood vessels (polyangiitis), which can restrict blood flow to various organs, and the formation of granulomas (masses of inflammatory tissue). It primarily affects tissues and organs that rely on smaller blood vessels, such as the upper respiratory tract, lungs and kidneys, but can involve other organs in the body as well.
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GPA is relatively rare, affecting approximately 3 per 100,000 people in the United States.
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It is more common in Caucasians and is slightly more common in males.
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It typically presents between ages 40 and 65, though it can occur at any age.
| Name | Abbreviation |
|---|---|
| Wegener’s Granulomatosis (historic term) | |
| Necrotizing Granulomatous Vasculitis | |
| ANCA-associated vasculitis (AAV), specifically PR3-ANCA associated vasculitis |
The exact cause of GPA remains unknown. It is considered an autoimmune disorder where the immune system mistakenly attacks the body's own tissues. There could be a genetic predisposition and environmental exposures or infections may possibly contribute. GPA is often associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA), particularly those targeting the enzyme proteinase 3 (PR3).
Symptoms can vary widely depending on the organs involved. Common symptoms include:
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Nasal congestion, runny nose, and nosebleeds
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Sinus pain and infections
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Headaches or pressure due to sinus congestion
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Cough and shortness of breath
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Chest pain
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Kidney problems (blood in the urine, protein in the urine)
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Joint pain
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Fatigue
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Fever
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Weight loss
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Skin lesions
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Neurologic symptoms such as numbness or weakness
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Ear problems, including hearing loss
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Eye inflammation.
| Name | Description |
|---|---|
| Nose symptoms | Pain and stuffiness in the nose |
| Hearing loss | Hearing loss |
| Conjunctivitis | Conjunctivitis |
Diagnosis is based on clinical presentation, laboratory tests and tissue biopsy. Key diagnostic features include:
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Presence of ANCA (anti-neutrophil cytoplasmic antibodies) in blood, particularly PR3-ANCA
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Urinalysis to detect kidney involvement (blood, protein in urine)
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Chest X-rays or CT scans to assess lung involvement
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Biopsies from affected tissues showing granulomatous or vascular inflammation
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Pulmonary function tests
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Kidney function tests.
Treatment aims to control inflammation and prevent organ damage. Common treatments include:
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Corticosteroids (e.g., prednisone)
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Immunosuppressant drugs (e.g., rituximab, cyclophosphamide, methotrexate)
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Plasma exchange (plasmapheresis) in cases of severe kidney involvement or pulmonary hemorrhage.
Before effective treatment, GPA was almost universally fatal. With modern immunosuppressive therapy, 80-90% of patients achieve remission. Long-term survival rates have significantly improved, with a 5-year survival rate exceeding 80%. GPA is a chronic condition. Relapses are common (occurring in over 50% of cases), requiring ongoing monitoring and management. Long-term complications can include kidney damage, hearing loss and respiratory problems.
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Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M. (2016). “Clinic manifestations in granulomatosis with polyangiitis.” Int J Immunopathol Pharmacol. 2:151-9. doi: 10.1177/0394632015617063. Epub 2015 Dec 18. PMID: 26684637; PMCID: PMC5806708.
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National Organization for Rare Disorders (NORD): Granulomatosis with polyangiitis.
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Orphanet: Granulomatosis with polyagniitis.
Last updated 6 Jun 2009, 10:50 PM
6 Jun 2009, 10:50 PM
I have had WG for 5 years now on Methatrexate inj, Bactrim, and of course prednisone. Recently I was diagnosed with Invasive Ductal Carcinoma. I had a Lumpectomy (left breast) and now I am preparing for Aftercare Treatment. I will have radiation, Arrmidex, and inj Zometa. My Oncologist has asked me if I feel I need chemo and it is my decision one way or the other. Has anyone out there had both diseases at same time already on maitaninace Chemo and now need to decide whether to go on a cytoxin???? What meds were you and what decisions did you make?? I would value your opinion and sharing of this serious matter. Judi
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After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.
Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.
Visit sanfordresearch.org/CoRDS to enroll.
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Adjuvant Therapy
Created by mucjud13 | Last updated 6 Jun 2009, 10:50 PM
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