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Diseases & Conditions

Wegener's Granulomatosis

What is Wegener's granulomatosis?

Wegener's granulomatosis (WG) is a rare disease of uncertain cause. It is characterized by inflammation in a variety of tissues, including blood vessels (vasculitis). Inflammation damages vital organs of the body.

WG primarily affects the upper respiratory tract (sinuses, nose, trachea [upper air tube]), lungs, and kidneys. Any other organ in the body can be affected as well.

Who is affected by the disease?

WG can occur at any age. The peak age groups affected are from 40-60 years. It affects men and women equally.

What are the symptoms?

The symptoms of WG and their severity vary among patients. General signs of the disease may include:

  • Loss of appetite
  • Weight loss
  • Fever
  • Fatigue

Most patients first notice symptoms in the respiratory tract. Symptoms may include:

  • Persistent runny nose (also called rhinorrhea) or the formation of nasal crusts and sores
  • Nasal or facial pain
  • Nose bleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
  • Cough that might include bloody phlegm caused by upper airway or lower airway (lung) inflammation
  • Chest discomfort with or without shortness of breath
  • Middle ear inflammation (also called otitis media), pain, or hearing loss
  • Voice change, wheezing, or shortness of breath caused by inflammation of the trachea

Other possible symptoms include:

  • Eye inflammation and/or bulging, with or without loss of vision
  • Joint pain (arthritis) or muscle pain
  • Rashes or skin sores
  • Kidney inflammation*

*Although kidney inflammation is common, it is not usually associated with symptoms, such as pain.

How is the disease diagnosed?

WG has symptoms similar to a number of other disorders, which may make it difficult to diagnose. However, for the most effective and successful treatment, early diagnosis is critical.

It is the combination of symptoms, results of physical examinations, laboratory tests, X-rays, and sometimes a biopsy (sample) of affected tissue (skin, nose, sinus, lung, kidney or other sites) that together prove the diagnosis of WG. Following treatment, these factors are also critical in judging whether the disease is active or in remission.

A positive blood test for antineutrophil cytoplasmic antibodies (ANCA) can support a suspected diagnosis of the disease. However, this blood test does not by itself prove the diagnosis of WG or determine disease activity.

To help your doctor judge disease activity, he or she may order:

  • A red blood cell count (to look for signs of anemia)
  • Sedimentation rate (the speed in which blood cells settle in a vertical glass tube)
  • Urinalysis
  • Chest or sinus X-rays

Sometimes the lungs may be abnormal even though there are no symptoms such as cough or shortness of breath. If symptoms of WG are apparent, but not from the lungs, one quarter to one third of patients may still have unexpected lung abnormalities detected on imaging tests (conventional X-rays or a CT scan). Therefore, it is important to have lung images performed if active WG is suspected -- even if you don't have any symptoms of lung disease.

How is the disease treated?

Because WG is often a life-threatening disease, it is treated with a variety of powerful drugs that have been shown to be life-saving.

Treatment usually includes corticosteroid medicines, such as prednisone, and other drugs that also suppress immune function. These include cyclophosphamide, methotrexate, azathioprine, or mycophenolate mofetil. Such drugs usually induce remission (the complete absence of all signs of the disease). Recent studies have also shown that a biologic agent called rituximab is as effective as cyclophosphamide and does not have some of the toxic effects of that drug. Improvement with these therapies usually occurs within days to weeks. When WG is in remission, the dosage of prednisone is reduced or often completely stopped. Some patients do require a low dose of prednisone to sustain remission. The ideal duration of treatment with the other immunosuppressive medications is uncertain. However, it is well established that WG is associated with relapse that may be as high as 60% in the first year and 80% by the end of the second year after stopping treatment. It must be borne in mind that these therapies can provide excellent control of disease, but are not cures.

The old practice of using cyclophosphamide over extended periods of time is no longer considered the "standard of care." Most patients with severe disease can be placed into remission with cyclophosphamide and corticosteroids within 3-4 months. Switching from cyclophosphamide to either methotrexate or azathioprine is usually effective in sustaining improvement or remission. This approach is a major change in strategy that has spared most patients the risks of long-term cyclophosphamide therapy. Indeed many patients who have milder forms of WG can be treated without cyclophosphamide; being able to achieve remission with agents such as methotrexate, mycophenolate mofetil or rituximab.

The treatment used for WG has also been successfully applied to other vasculitic diseases.

What are the side effects of treatment?

Because these drugs suppress the immune system, there is an increased risk of developing serious infections. Prednisone can also cause weight gain, cataracts, brittle bones, high blood pressure, diabetes, and changes in mood and personality. Cyclophosphamide can cause sterility, bladder irritation and bleeding, and even cancer of the bladder when used over extended periods of time. Thus every effort is made to control severe disease with cyclophosphamide and prednisone and then stop cyclophosphamide after about 3-6 months and substitute other agents that may be effective, although are less powerful. Methotrexate can cause liver irritation. Cyclophosphamide and methotrexate can each cause changes in blood counts and sometimes lung inflammation.

Because the medicines used to treat WG can have serious side effects, patients are monitored closely by their doctor. The dosage of medicine is adjusted as needed throughout the course of treatment.

The risk of side effects has stimulated research to discover other effective treatments that have less risk. An excellent example is rituximab, an agent recently demonstrated to be as effective as cyclophosphamide. Experience with this new therapy is far less than the others noted above. The precise role that it will play in treatment of WG is still being defined.

Because all of these drugs have some risk, laboratory tests that may detect side effects are regularly monitored in patients with WG. These tests, although not perfect, may also reflect worsening of disease activity, even in patients that feel well. Thus, regular monitoring checks are important for medication tolerance and are also one measure of disease activity.

What is the outlook for people with Wegener's granulomatosis?

After treatment and improvement occurs, relapses of the disease are common, especially if maintenance treatments are fully withdrawn. Flare-ups might follow reductions of the doses of prednisone, methotrexate, azathioprine, or cyclophosphamide. Flare-ups can usually be controlled by increasing the dose of these medicines.

WG is a very serious disease, and its treatment carries significant risks. However, treatment is life-saving for almost everyone when the diagnosis is made in a timely fashion and appropriate treatment instituted. This includes regular monitoring of disease activity and possible medication toxicity.

Prior to recognizing effective therapy in the 1970s, half of all patients with this illness died within 5 months of diagnosis. Today, more than 75% of treated patients are alive at least eight years later. For many people with WG, long term survival has been seen with many also being able to lead relatively normal lives. If past is prelude to the future, ongoing research will lead to further discoveries and even better treatment.

References

Vasculitis Foundation.
Wegener’s Granulomatosis.
www.vasculitisfoundation.org/wegenersgranulomatosis.
Accessed 9/30/2010

Schilder AM. Wegener's Granulomatosis vasculitis and granuloma. Autoimmun Rev 2010;9:483–487

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 10/21/2010…#4757

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