Wegener's Granulomatosis

Abstract

Wegener's granulomatosis (WG) is a form of noninfectious necrotizing systemic vasculitis which commonly involves the upper airways, lungs, and kidneys. Disease manifestations are variable, making accurate diagnosis and distinction from other forms of systemic vasculitis difficult in some cases. A careful clinical, laboratory, and pathologic evaluation, including assessment for the presence of antineutrophil cytoplasmic 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 methotrexate may also be useful to induce and/or maintain remission.

Key Words:

Wegener's granulomatosis - vasculitis - antineutrophil cytoplasmic antibodies (ANCA)