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- Wegener's granulomatosis is due to an inflammation of the small blood vessels (vasculitis) and formation of granulomas (collection of cells) in the body. This disease occurs in both men and women, usually affecting people 40 to 50 years old. Without treatment, all patients with this disease will die.
- The disease most often affects the lungs and kidneys.
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- Almost all patients with this disease have problems such as congestion of the nose, ear infections, sinus infections, or they develop large gums. Many also develop coughs, have trouble breathing, or they cough-up blood.
- Fever, night sweats, sores, or ulcers may form around the nostrils, while granulomas may block the nasal passages.
- Other symptoms include one eye protruding out, Bruising, and joint pain.
- This disease will eventually affect the kidneys of most patients, and can very quickly cause kidney failure. Some patients may notice blood in their urine.
- Some patients will have a "saddle" shaped nose due to the disease destroying parts of the nose.
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- This disease is caused by an inflammation and destruction of the small blood vessels in the body by the person's own immune system. However, it is not known why the immune system attacks the person's own blood vessels.
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- Diagnosis is made by a biopsy, which involves obtaining a piece of tissue from the effected organ, and looking at it under the microscope. The most common areas that undergo a biopsy are the lungs, kidney, or nose.
- Blood tests for ANCA (antineutrophil cytoplasmic antibody) are usually positive in patients with Wegener's granulomatosis.
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- The goal is to make the diagnosis early and begin treatment before there is kidney failure.
- The primary treatment is with the medicines cyclophosphamide (an anti-cancer drug) and prednisone (a steroid). The disease can be stopped in up to 75% of patients treated with these medicines. However, up to half of the patients who are successfully treated will eventually develop the disease again.
- Also, there is an increased risk of developing cancer in those patients treated with cyclophosphamide.
- Other drugs such as Methotrexate (an anti-cancer drug) and trimethoprim-sulfamethoxazole (an antibiotic) can be used to prevent repeat flare-ups of the disease.
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