La Revue du praticien
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La Revue du praticien · Mar 2008
Review Comparative Study[Wegener's granulomatosis and microscopic polyangiitis].
Wegener's granulomatosis and microscopic polyangiitis are among the main systemic necrotizing vasculitides predominantly affecting small vessels. Their prevalences range from 24 to 157 cases per million inhabitants. Mean age at onset is usually 40 to 60 years old. ⋯ Therapy relies on the combination of corticosteroids and pulse intravenous cyclophosphamide, which can be switched, as soon as remission is achieved, to azathioprine or methotrexate, for a total duration of treatment of at least 18 months. Ten-year survival rate now exceeds 80%, but relapses are frequent. The precise place of new biologics, such as rituximab, needs to be further defined.
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The vasculitides involving the lower respiratory tract are mainly the small vessels vasculitides associated with antineutrophil cytoplasmic autoantibodies including Wegener granulomatosis (WG), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (SCS). WG pulmonary manifestations mainly consist of nodules and pneumonias often cavitary, and tracheobronchial stenoses. ⋯ In SCS, asthma with eosinophilia and further eosinophilic pneumonia are the characteristic pulmonary features. Lung biopsy may be necessary in WG to obtain a definite diagnosis when no other site for biopsy is possible.