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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How do you compare to others with this condition?

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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 624 free, full-text research articles on human participants. First 3 results:

(18)F-FDG PET imaging of granulomatosis with polyangiitis -Wegener's Syndrome.
 

Author(s): Frank De Geeter, Pieterjan Gykiere

Journal: Hell J Nucl Med. ;19(1):53-6.

 

Granulomatosis with polyangiitis (GPA), formerly called Wegener's syndrome, is a vasculitis associated with antineutrophil cytoplasmic antibody and may occur in all ages but mostly in order adults. The organs most frequently involved are the ear, nose and throat (rhinitis, sinusitus, ...

Last Updated: 2 Apr 2016

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Granulomatosis with polyangiitis, a new nomenclature for Wegener's Granulomatosis--Case report.
 

Author(s): Alexandre Moretti de Lima, Pedro de Freitas Silva Torraca, Sheila Pereira da Rocha, Carmelia Matos Reis Santiago, Fabio Humberto Ribeiro Paes Ferraz

Journal: An Bras Dermatol. ;90(3 Suppl 1):101-3.

 

The granulomatosis with polyangiitis, initially known as Wegener's granulomatosis, is a small and medium vessels vasculitis. It's classic form presents a triad: necrotizing granuloma of respiratory tract, necrotizing cutaneous vasculitis and glomerulonephritis. This vasculitis has ...

Last Updated: 28 Aug 2015

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Tracheobronchial Stenoses in Granulomatosis With Polyangiitis (Wegener's): A Report on 26 Cases.
 

Author(s): Charlotte Girard, Pierre Charles, Benjamin Terrier, Guillaume Bussonne, Pascal Cohen, Christian Pagnoux, Vincent Cottin, Jean-François Cordier, Loïc Guillevin

Journal: Medicine (Baltimore). 2015 Aug;94(32):e1088.

 

Tracheobronchial stenoses (TBSs) are potentially severe manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) that usually respond poorly to corticosteroids and immunosuppressive agents. We describe 26 GPA patients with ≥1 tracheal (mainly subglottic, SGS) and/or ...

Last Updated: 13 Aug 2015

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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 37 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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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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Cyclophosphamide as induction therapy for Wegener's granulomatosis and microscopic polyangiitis.
 

Author(s): C A Langford

Journal: Clin. Exp. Immunol.. 2011 May;164 Suppl 1():31-4.

 

The introduction of cyclophosphamide (CyP) as a treatment for Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) has been among the most significant contributions in vasculitis. Prior to the introduction of CyP, WG was a uniformly fatal disease, with mortality occurring ...

Last Updated: 30 Mar 2011

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

 
 
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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: 5 Jul 2016

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Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis
 

Status: Not yet recruiting

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

 

Last Updated: 16 Dec 2015

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Last Updated: 1 Nov 2016

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