WEGENERS GRANULOMATOSIS

Wegener's granulomatosis (WG) is a form of noninfectious necrotizing s ystemic vasculitis which commonly involves the upper airways, lungs, a nd kidneys, Disease manifestations are variable, making accurate diagn osis and distinction from other forms of systemic vasculitis difficult in some cases, A careful clinical, laboratory, and pathologic evaluat ion, including assessment for the presence of antineutrophil cytoplasm ic antibodies (ANCA) may be helpful. Without treatment the disease is usually fatal, However, remission is common in those patients treated with a combination of prednisone and cyclophosphamide. Recent studies have also suggested that other cytotoxic agents such as weekly methotr exate may also be useful to induce and/or maintain remission.