Wegener's granulomatosis

Common Name(s)

Wegener's granulomatosis, Granulomatosis with polyangitis

Granulomatosis with polyangitis (GPA), or Wegener’s granulomatosis (WG), is a rare condition that causes inflammation in the blood vessels. In addition to inflammation, GPA also causes the development of abnormal tissue, called granulomas, which can destroy normal tissue. This combination of inflamed blood vessels and destruction of normal tissue causes poor blood flow to organs, leading to permanent organ damage. The upper respiratory system, lungs, and kidneys are the areas most commonly affected by GPA. The first symptoms of this condition can include nosebleeds, runny nose with pus-filled discharge, ear infections, chest pain, shortness of breath and coughing up blood. Other symptoms may include unintended weight loss, blood in the urine, skin sores, eye redness, or fever.

The cause of GPA is unknown but researchers believe GPA develops after an initial inflammation-causing event leads to an abnormal immune system reaction. GPA can affect anyone, but most people get GPA between the ages of 45 and 80.

In addition to taking a detailed medical history and performing a physical exam, your doctor may conduct several tests to diagnose GPA. Blood tests may be used to check for a specific protein (autoantibody) that can be seen in people with GPA, called anti-neutrophil cytoplasmic autoantibodies (ANCA). Although many people with GPA have ANCAs in their blood, not everyone with GPA does. Also, ANCAs may be present in people who don’t have GPA. In addition to blood tests, your doctor may also perform urine tests (to check kidney function), chest x-rays, or they may remove and examine a small piece of tissue from an affected organ (biopsy). Treatment of GPA usually includes medications to slow down your immune system. Severe forms of GPA may require surgery. The earlier GPA is detected and treated, the more likely a person is to recover. Talk with your doctor about the most current treatment options. Support groups may also be helpful.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Wegener's granulomatosis" for support, advocacy or research.

stopcaidnow.org

Our mission is simple-Educate, Provide Awareness, and Fund Researchers who are committed to finding a cure. StopCAIDnow(501c3) is focused on providing tools to better educate Doctor’s and the community on AutoInflammatory diseases. There is a need for an understanding of these diseases so proper diagnosis can be made, and treatment can be started. This will help alleviate the inflammation process, 7 the pain that comes with it. Proper diagnosis is urgent for the prognosis of these diseases. If left untreated damage to the tissues, organs & vision(for some) will not only be irreversible but devastating

Last Updated: 5 Mar 2013

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General Support Organizations

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Wegener's granulomatosis" for support, advocacy or research.

stopcaidnow.org

Our mission is simple-Educate, Provide Awareness, and Fund Researchers who are committed to finding a cure. StopCAIDnow(501c3) is focused on providing tools to better educate Doctor’s and the community on AutoInflammatory diseases. There is a need for an understanding of these diseases so proper diagnosis can be made, and treatment can be started. This will help alleviate the inflammation process, 7 the pain that comes with it. Proper diagnosis is urgent for the prognosis of these diseases. If left untreated damage to the tissues, organs & vision(for some) will not only be irreversible but devastating

http://www.stopcaidnow.org

Last Updated: 5 Mar 2013

View Details

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Wegener's granulomatosis" returned 534 free, full-text research articles on human participants. First 3 results:

Bilateral acute angle-closure glaucoma as a first presentation of granulomatosis with polyangiitis (Wegener's).
 

Author(s): Alper Mete, Sabit Kimyon, Oguzhan Saygili, Can Pamukcu, Kıvanç Güngör

Journal: Arq Bras Oftalmol. ;79(5):336-338.

 

We report a case of bilateral acute angle-closure glaucoma in a patient with undiagnosed granulomatosis with polyangiitis (Wegener's). A 59-year-old man presented with a severe headache, ocular pain, blurred vision, shortness of breath, and mild fever. Clinical examination revealed ...

Last Updated: 16 Dec 2016

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The utility of needle core biopsy in the diagnosis of Wegener's granulomatosis.
 

Author(s): Mona Mlika, Saoussen Hantous, Imen Helal, Emna Braham, Ali Mrabet, Faouzi El Mezni

Journal: Tunis Med. 2016 Mar;94(3):244-5.

 

Last Updated: 31 Aug 2016

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Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's): Distinct Patient Subsets.
 

Author(s): Eli M Miloslavsky, Na Lu, Sebastian Unizony, Hyon K Choi, Peter A Merkel, Philip Seo, Robert Spiera, Carol A Langford, Gary S Hoffman, Cees G M Kallenberg, E William St Clair, Nadia K Tchao, Fernando Fervenza, Paul A Monach, Ulrich Specks, John H Stone

Journal: Arthritis Rheumatol. 2016 Dec;68(12):2945-2952.

 

To examine the relationship of antineutrophil cytoplasmic antibody (ANCA) type and ANCA-associated vasculitis (AAV) diagnosis with demographic features, disease manifestations, and clinical outcomes. We focused on patients who account for the differences between ANCA type and disease ...

Last Updated: 18 Jul 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Wegener's granulomatosis" returned 34 free, full-text review articles on human participants. First 3 results:

Wegener's granulomatosis and pyoderma gangrenosum--rare causes of facial ulcerations.
 

Author(s): Karolina Kędzierska, Krzysztof Sindrewicz, Tomasz Smektała, Magda Wiśniewska, Marek Masiuk, Ewelina Staniszewska, Katarzyna Sporniak-Tutak, Edyta Gołembiewska, Kazimierz Ciechanowski

Journal:

 

Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG). The purpose of this article was to compare ...

Last Updated: 27 Apr 2016

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[Computed tomography findings in pulmonary involvement of Wegener's granulomatosis: pictorial review].
 

Author(s): Necdet Poyraz, Celalettin Korkmaz, Durdu Mehmet Yavşan, Suat Keskin, Turgut Teke, Kemal Ödev

Journal: Tuberk Toraks. 2015 ;63(4):257-64.

 

Wegener's granulomatosis is a necrotising granulomatous vasculitis which has a variable manifestations in the chest that are best described on computed tomography. Imaging findings may include masses or nodules, which may cavitate; consolidations and ground-glass opacities. Wegener's ...

Last Updated: 11 Mar 2016

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Panhypopituitarism due to Wegener's granulomatosis.
 

Author(s): Silvina G Santoro, Alberto H Guida, Alejandra E Furioso, Patricia Glikman, Amelia S Rogozinski

Journal: Arq Bras Endocrinol Metabol. 2011 Oct;55(7):481-5.

 

Wegener's granulomatosis (WG) is a multi-system necrotizing granulomatous vasculitis which classically affects the upper respiratory tract, lungs and kidneys. Pituitary participation has been described in 24 patients in the literature to date. The aim of this article is to report ...

Last Updated: 7 Dec 2011

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Neutrophils as Prognostic Factors in Granulomatosis With Polyangiitis (Formerly Named Wegener's Granulomatosis)
 

Status: Recruiting

Condition Summary: Granulomatosis With Polyangiitis

 

Last Updated: 15 Dec 2016

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Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis
 

Status: Recruiting

Condition Summary: Granulomatosis With Polyangiitis (Wegener's Granulomatosis)

 

Last Updated: 20 Apr 2017

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Natural History of Granulomatosis With Polyangiitis: Clinical and Genetic Biomarkers of Airway Disease NoAAC PR-03 Study
 

Status: Not yet recruiting

Condition Summary: Granulomatosis With Polyangiitis

 

Last Updated: 13 Sep 2017

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