Clinical manifestations and treatment of Wegener's granulomatosis

Wegener's granulomatosis (WG) is characterized by granulomatous lesions and vasculitic disease manifestations. Granulomatous lesions are found in the upper and lower respiratory tract (eg, granulomatous sinusitis, orbital masses, and pulmonary granuloma), whereas vasculitic manifestations occur frequently in lung (alveolar hemorrhage) and kidney (glomerulonephritis). Vasculitis is typically associated with antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3. WG has been traditionally associated with a poor outcome and increased mortality as documented by numerous studies; however, recent cohort studies report an improved outcome, probably a consequence of increased awareness leading to an earlier diagnosis, and to improved treatment strategies derived from evidence from controlled trials. Treatment regimens for WG, adapted to disease stage and activity, are reviewed and discussed in this article.