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Medical Author:

William C. Shiel Jr., MD, FACP, FACR

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.

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In this Article

• What is Wegener's granulomatosis?
• What are symptoms of Wegener's granulomatosis?
• How is Wegener's granulomatosis diagnosed?
• How is Wegener's granulomatosis treated?
• Wegener's Granulomatosis At A Glance
• Patient Comments: Wegener's Granulomatosis - Treatment
• Patient Comments: Wegener's Granulomatosis - Symptoms
• Wegener's Granulomatosis Index

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.

• Wegener's granulomatosis is an uncommon disease that involves inflammation of blood vessels (vasculitis).
• Symptoms of Wegener's granulomatosis include fatigue, weight loss, fevers, shortness of breath, bloody sputum, joint pains, and sinus inflammation.
• Diagnosis of Wegener's granulomatosis is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis.
• Treatment is directed toward stopping the inflammation process by suppressing the immune system.

REFERENCES:

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.