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Q: What is Wegener's disease?
A: Wegener's disease, also known as Wegener's granulomatosis, is a disease which involves primarily the respiratory tract, but which also commonly affects the kidneys and arteries. The lungs are usually affected and the typical presentation of this disease consists of several round or oval lesions in the lungs, which may be visualized on chest X-ray. These lesions may have cavities within them, although this is not always necessarily the case. In view of these lesions, patients frequently present with cough, shortness of breath, coughing of blood (hemoptysis). Lesions in the nose and pharynx may also occur and patients frequently present with sinusitis. In addition, destruction of the cartilage of the nose may give rise to a saddle nose deformity. Also, middle ear inflammation, as well as hearing loss are common in the disease. Although the kidneys are affected in about 85% of the cases, their involvement usually does not cause symptoms before the diagnosis is established. Approximately 50% of patients with this disease have involvement of the eyes, about 30% have cardiac involvement, and about 25% have involvement of the nervous system. Almost 50% of the patients have skin manifestations. Fever, feeling bad, and weight loss are common.
The diagnosis is definitely made on lung biopsy. Typical pathologic change consists in the finding of the so called necrotizing granulomatous lesions. In other words, destructive, cavitary lesions (referred to as necrotizing lesions) are found. Furthermore, these lesions are also inflammatory in character, are ovally shaped or round, and prominently exhibit various types of inflammatory cells ( in particular, the so called eosinophils, plasma cells, cells with one nucleus (mononuclear cells), and giant cells). This type of lesions is referred to as granulomatous. A large number of patients with this disease have a typical antibody in the blood, the so called antineutrophil cytoplasma antibody (ANCA). In a patient with typical clinical findings, a biopsy that is suggestive (from skin, nose, etc.), and a positive ANCA test, the diagnosis can be made with a high degree of probability, even in the absence of a lung biopsy.
It is important that the diagnosis is made early, since the disease invariably leads to death if not treated in the early stages. Particular success has been achieved with cyclophosphamide and prednisone. In addition, methrotexate is as effective as cyclophosphamide in patients who do not have immediate life-threatening disease, thus replacing cyclophosphamide as the initially usually employed treatment. Finally, bactrim has been shown to be effective in trying to prevent the reocurrence of the disease in patints who are disease free.
Updated: 06/28/99
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