[Wegener's granulomatosis: diagnosis and treatment]

Wegener's granulomatosis (WG) is a systemic, necrotizing and granulomatous vasculitis that affects the upper and lower respiratory tract and the kidney. It is the most common antineutrophil cytoplasmic antibodies (ANCA) vasculitis. The diagnosis of WG is based on clinical manifestations, histological findings and the presence of ANCA in serum. The American College of Rheumatology criteria includes: oral and nasal inflammation, abnormal chest radiography (nodules, fixed infiltrates, or cavities, urinary sediment (hematuria), and granulomatous inflammation on biopsy. Treatment of WG is divided into 2 phases, induction of remission (IR), followed by a maintenance phase (MP). In the IR it is necessary to use immunosuppressive intensive therapy (cyclophosphamide plus steroids) and the MP is a less intensive therapy in which immunosuppressors (IS) such as azathioprine, methotrexate, mycophenolate of mofetil, among others, may be employed. Their purpose is to keep remission and lower the adverse effects associated with IS. Etanercept has not been proven successfully for the MP. Rituximab and 15-dexopergualin constitute promising the rapies for refractory WG.