The pulmonary vasculitides are a heterogeneous group of rare disorders that result from an inflammatory process damaging the vessel wall and consequent impaired blood flow, ischaemia and tissue necrosis. The clinical manifestation of these vasculitides depends on the site, size, type and severity of the inflammatory process. Vasculitis involving the airways is a common feature of the anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitides (AASVs) and can predate the diagnosis by years. Secondary causes of vasculitis associated with connective tissue disorders are also capable of presenting with pulmonary features. Recognition of involvement, investigation and treatment are important to ameliorate symptoms for patients. This article concentrates on the assessment and specific management of upper and lower airway problems of AASV.