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All About Wegener’s Granulomatosis: Symptoms, Diagnosis, Treatment and Research Toward a Cure

written by: bjlbyron • edited by: Leigh A. Zaykoski • updated: 8/17/2010

Wegener's Granulomatosis (WG) is an inflammatory disease that affects about 1 in every 20,000-30,000 individuals. This article discusses the symptoms of WG, the methods for diagnosing and treating it, and current research that is being carried out toward finding a cure.

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    What are the symptoms of WG?

    At first, WG sufferers typically, but not always, experience cold-like symptoms, such as chronic runny nose, sinus discomfort or pain, coughing, fever, fatigue, and general malaise.

    The area of the body that is most commonly affected among WG patients is the upper respiratory tract, which includes the larynx, pharynx and nasal cavity (sinuses). In addition to some of cold-like symptoms listed above, symptoms specific to the upper respiratory tract include upper respiratory tract discomfort, frequent nosebleeds, and sinus ulcers. In severe cases, these upper respiratory tract ailments lead to hearing loss or even deafness and to defects in the structure of cartilage in the nose, which can cause the nose to deform and to even collapse in some cases.

    Other areas of the body that can be affected by WG include the lungs, the muscular system, the skeletal system, the kidneys, the skin, and the eyes. The most serious symptoms specific to these areas are presence of blood in the lungs (which typically gets coughed up and expelled through the mouth by the sufferer), eye lesions, decreased vision, and in the very worst cases, kidney malfunction or failure.

    If left untreated, WG can cause death, especially in individuals whose kidneys are severely affected. However, when WG is detected early, death rarely results.

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    How is WG diagnosed?

    The only known definitive way to diagnose WG is by microscopic detection of vasculitis (blood vessel swelling) and granulomas (mass of cells) in tissue material removed from the patient by biopsy. However, since WG symptoms are common to a number of ailments, medical professionals typically do not suspect right away that WG is the causative disease, and therefore biopsy followed by microscopic inspection usually follows one on more other tests that can suggest that WG is the cause of a patient's ills. These tests include x-rays, which can show lung problems brought on by WG, blood tests for certain proteins that are indicative of WG, and urine tests, which can reveal kidney malfunction.

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    What treatment options are available to WG patients?

    Although the symptoms of WG can be discomforting, debilitating and life-threatening, the long-term prognosis is very good for many WG patients as a number of drugs that effectively alleviate WG symptoms are available. These drugs include cyclophosphamide, azathioprine, methotrexate and prednisone, among others. One significant limitation of these drugs is that they can cause severe side effects, which sometimes can be avoided or reduced through routine monitoring of the patient. Another drawback is that about half of all WG patients who are initially effectively treated experience a recurrence of the disease after treatment is ceased. For these reasons, researchers are trying to develop a cure that is free of any serious side effects and that minimizes the likelihood of recurrence.

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    What research is being done toward finding a cure for WG?

    It is strongly suspected that Wegener's Granulomatosis has an underlying genetic basis. For this reason, much WG research is focused on finding a genetic cause, knowledge of which could be used to develop a treatment that could effectively prevent people from being inflicted with the disease and/or could eradicate the condition in individuals who suffer from WG. Although the gene or genes responsible for the disease have not been identified, recent research efforts have narrowed the area of the genome in which the culprit gene resides (or the genes reside) to a very small portion. It is therefore expected that the genetic cause of WG soon will be found. Based on knowledge of the gene's function (or the genes' functions), it is hoped that scientists will be able to develop agents for optimally treating WG without side effects and without risk of recurrence.

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    References

    F. Moosig et al. Wegener's granulomatosis: the current view. Clin Rev Allergy Immunol. Oct 2008;35(1-2):19-21.

    S. Wieczorek et al. Recent progress in the genetics of Wegener's granulomatosis and Churg-Strauss syndrome. Curr Opin Rheumatol. Jan 2010;22(1):8-14.

    Wegener's Granulomatosis: Definition, The Mayo Clinic. http://www.mayoclinic.com/health/wegeners-granulomatosis/DS00833

    Wegener's Granulomatosis, National Institute of Allergy and Infectious Diseases. http://www.niaid.nih.gov/TOPICS/WEGENERS/Pages/symptoms.aspx

    Wegener's Granulomatosis, Vasculitis Foundation. http://www.vasculitisfoundation.org/wegenersgranulomatosis