[Wegener's granulomatosis].

Wegener's granulomatosis is characterized by 3 major lesions: necrosis, granulomatosis, and vasculitis. Although all organs may be involved, chronic rhinosinusitis, pulmonary involvement (especially with the typical imaging pattern of cavitary nodules), and glomerulonephritis are the most common clinical manifestations. Antineutrophil cytoplasmic autoantibodies (characteristically cytoplasmic with anti-proteinase 3 specificity) are of considerable help for diagnosis. Remission of disease is obtained with corticosteroids and cyclophosphamide, but relapses are frequent, and treatment toxicities are a major concern.