Update on the Management of Respiratory Manifestations of the Antineutrophil Cytoplasmic Antibodies-Associated Vasculitides

Antibody-associated vasculitis comprises 3 small vessel vasculitis syndromes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic polyangiitis (EGPA). This article outlines the major tracheobronchial and pulmonary parenchymal disease manifestations of GPA and MPA and their management, as well as relevant recent advances in the treatment of EGPA. Shared trends in the management of all 3 syndromes are: (1) a focus on glucocorticoid avoidance and (2) an increasing reliance on biologic agents. Evidence from randomized controlled trials and large cohort studies in support of these trends as well as ongoing research efforts are summarized.