Management of Wegener Granulomatosis

Abstract

Wegener granulomatosis (WG) is the most common of the pulmonary granulomatous vasculitides. Organ involvement characteristically includes the upper and lower respiratory tracts and kidney, but virtually any organ can be involved. Severity of WG varies greatly among patients, ranging from indolent, chronic disease to fulminant, fatal vasculitis. Similarly, the degree of organ involvement differs among patients; WG may involve only a single organ (typically the lungs or upper respiratory tract) or may involve multiple organs. Histologically, WG is defined by necrotizing vasculitis involving small vessels, "geographic" necrosis, and granulomatous inflammation. Therapeutic regimens for active WG are generally effective at inducing remissions, but relapses after discontinuation of therapy are common. Early, short-course induction treatment with cyclophosphamide and corticosteroids for generalized, multiorgan WG, followed by maintenance therapy with less toxic agents (eg, methotrexate, azathioprine) is recommended. Recent studies also suggest that methotrexate combined with corticosteroids may be adequate for inducing remission in non-life-threatening WG. For tracheobronchial/subglottic stenosis and for orbital involvement, alternative therapeutic modalities are discussed.