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

Granulomatosis with polyangiitis (Wegener's) as a necrotizing gingivitis mimic: a case report.
 

Author(s): Katrina Genuis, Jesse Pewarchuk

Journal:

 

Granulomatosis with polyangiitis poses a significant diagnostic dilemma due its diverse presentations. Seemly isolated sites of disease, such as oral ulcers, may present to physicians working in primary care settings, the emergency room, and subspecialty fields as well as to dentists. ...

Last Updated: 20 Sep 2014

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Cytoplasmic-anti-neutrophil cytoplasmic antibodies targeting myeloperoxidase in Wegener's granulomatosis: a rare phenomenon.
 

Author(s): Bhavana M Venkatesh, Sangeeta Joshi, Ranjeeta Adhikary

Journal: Indian J Pathol Microbiol. ;57(3):470-2.

 

Wegener's granulomatosis (WG) patients can rarely have antineutrophil cytoplasmic antibodies (ANCAs) directed against myeloperoxidase (MPO), producing a cytoplasmic pattern on indirect immunofluorescence (IIF). This has important implications in the diagnosis and pathophysiology of ...

Last Updated: 14 Aug 2014

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Isolated primary pancreatic Wegener's granulomatosis: report of two cases.
 

Author(s): Elissaios Kontis, Niovi Papalexopoulou, Yo Zen, Andreas A Prachalias

Journal:

 

Chronic inflammatory vasculitis, namely Wegener's granulomatosis, is an autoimmune disease predominantly affecting the respiratory tract and rarely the mucosa of the upper or lower gastrointestinal tract. We report two cases of isolated pancreatic manifestation of Wegener's granulomatosis, ...

Last Updated: 31 Jul 2014

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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:

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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Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener's granulomatosis).
 

Author(s): J C Jennette

Journal: Clin. Exp. Immunol.. 2011 May;164 Suppl 1():7-10.

 

Names influence how something is perceived. Diagnostic terms (diagnoses) are the names of diseases that are usually derived either from some distinctive characteristic of the disease or include an eponym recognizing someone who elucidated the disease. No matter how logical and appropriate ...

Last Updated: 30 Mar 2011

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's)
 

Status: Recruiting

Condition Summary: Granulomatosis With Polyangiitis (Wegener's); Granulomatosis With Polyangiitis; Wegener's Granulomatosis; ANCA-Associated Vasculitis

 

Last Updated: 23 Jul 2015

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Last Updated: 1 Jul 2015

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

Status: Recruiting

Condition Summary: Granulomatosis With Polyangiitis

 

Last Updated: 29 Apr 2015

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