Wegener's Granulomatosis
What is Wegener's granulomatosis?
Wegener's granulomatosis is a uncommon type of inflammation of small arteries and veins (vasculitis). It classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages (sinuses), and the kidneys. "Incomplete" forms exist that only involve one of these areas. When both lungs and kidneys are affected, the condition is sometimes referred to as generalized Wegener's granulomatosis. When only the lungs are involved, the condition is sometimes referred to as limited Wegener's granulomatosis.
Wegener's granulomatosis usually affects young or middle-aged adults. Although it is uncommon in children, it can affect people at any age. The cause of Wegener's granulomatosis is not known.
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 blood 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 which 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, 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 (cyclophosphamide) that is taken by mouth with prednisone until the disease is in remission and then switched to methotrexate for 2 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. 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 2 years. This is good news for patients with Wegener's granulomatosis.
Wegener's Granulomatosis At A Glance
- 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.
Author: William C. Shiel Jr., MD, FACP, FACR
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