Patients with ANCA-associated vasculitis (granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) present with a wide range of respiratory tract manifestations including pulmonary nodules, cavitary masses and infiltrates, tracheobronchial inflammation and stenosis, and diffuse alveolar hemorrhage. These patients require an early diagnosis and effective multidisciplinary management to prevent irreversible damage and carefully quided immunosuppressive therapy and often also airway procedures to maintain or restore airway patency.This presentation reviews the various disease manifestations, a systemic diagnostic approach to the patients with GPA or MPA and respiratory symptoms as well as treatment options in light of recent trial results.