[Wegener's granulomatosis: classification and therapy].

Wegener's granulomatosis in its classic form is manifested by necrotizing granulomas of the upper and lower respiratory tract, focal necrotizing vasculitis involving both arteries and veins which may be widely disseminated, and focal necrotizing glomerulitis. The disease may present with incomplete expression involving any combination of the major sites, including the upper respiratory tract (E), the lung (L), or the kidney (K). A newly emerging test for the disease called antineutrophil cytoplasmic antibody (ANCA) shows promise in studying the disease in its various expressions. Standard current treatment includes glucocorticoids and cyclophosphamide. Recently, favorable reports of improvement on trimethoprim/sulfamethoxazole combination have appeared, suggesting the possibility that the disease may be incited by a microbial infection.