Wegener's Granulomatosis (cont.)
William C. Shiel Jr., MD, FACP, FACR
Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
In this Article
- Wegener's granulomatosis facts
- What is Wegener's granulomatosis?
- What are symptoms of Wegener's granulomatosis?
- How is Wegener's granulomatosis diagnosed?
- How is Wegener's granulomatosis treated?
What are symptoms of Wegener's granulomatosis?
Symptoms of Wegener's granulomatosis include fatigue, weight loss, fevers, shortness of breath, bloody sputum, joint pains, and sinus inflammation (sinusitis). Nasal ulcerations and even bloody nasal discharge can occur. Other areas of the body that can also become inflamed in patients with Wegener's granulomatosis include the eyes, the nerves(neuropathy), the middle ear (otitis media), and the skin resulting in skin nodules or ulcers.
How is Wegener's granulomatosis diagnosed?
Abnormal lab findings in patients with Wegener's granulomatosis include urine tests that detect protein and red blood cells in the urine (not visible to the naked eye) and x-ray tests of the chest and sinuses which detect abnormalities resulting from lung and sinus inflammation. Blood tests that detect the abnormal inflammation include the sedimentation rate (sed rate) and C-reactive protein. A more specific blood test used to diagnose and monitor Wegener's granulomatosis is the antineutrophil cytoplasmic antibody (ANCA test), which is commonly elevated when the disease is active.
The diagnosis of Wegener's granulomatosis is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis in a biopsy of tissue involved with the inflammatory process. For examples, an open lung biopsy or a kidney biopsy are commonly used in making a diagnosis of Wegener's granulomatosis.
How is Wegener's granulomatosis treated?
Wegener's granulomatosis is a serious disease and without treatment can be fatal within months. Treatment is directed toward stopping the inflammation process by suppressing the immune system.
Medications used to treat Wegener's granulomatosis include high-dose cortisone(prednisone) and the immunosuppressive drug cyclophosphamide (Cytoxan). Recent reports also suggest that trimethoprim/sulfamethoxazole (Bactrim) can also be helpful to prevent relapse of disease activity in patients with Wegener's granulomatosis.
Cytoxan that is taken by mouth with prednisone until the disease is in remission and then switched to methotrexate for two years and tapered off has been reported to be effective and less toxic than the traditional long-term Cytoxan treatment.
Methotrexate has recently been introduced as a drug for Cytoxan treatment failures. Moreover, it now appears that Cytoxan will not be necessary in order to maintain long-term remission and that doctors can convert to the less toxic methotrexate for maintenance. The reports also demonstrate that methotrexate can eventually be tapered off entirely after two years. Azathioprine (Imuran) has also been used as a maintenance medication after Cytoxan. Recently, intravenous immunoglobulin therapy (IVIG) has been shown to be helpful in treating relapses of Wegener's granulomatosis. Also, preliminary studies suggest that rituximab (Rituxan) may be helpful to maintain remission once the initial inflammatory disease has been controlled using medications mentioned above. These new regimens are welcome news for patients with Wegener's granulomatosis as medical researchers are searching for better treatments.
Medically reviewed by Paul Skudder, Jr., MD; American Board of Surgery with subspecialties in Vascular Surgery and Surgical Critical Care
Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.
Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et al., 2000.American College of Rheumatology National Meeting, November 2005, 2006, 2007.
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