The lung is frequently involved in primary vasculitis especially in Wegener’s granulomatosis. Pulmonary involvement can occasionally occur alone but is more commonly seen as part of a more generalised disease. The eyes, ears, nose, sinuses, oral cavity and salivary glands are other common targets of injury. The trachea and bronchi may be affected with inflammatory pseudotumour leading to subglottic stenoses of the trachea and bronchial stenoses are well recognised. Renal involvement is frequently found and only rarely occurs without evidence of other organ involvement. The skin, joints, central and peripheral nervous system, gastrointestinal tract, genital tract, spleen, and heart may all be targeted in the inflammatory process. Pulmonary involvement per se may give rise to solitary or multiple nodules which often cavitate and can be surrounded by thick walls of inflammatory tissue. Localised or diffuse pulmonary infiltrates, lobar collapse, atelectasis, and alveolar haemorrhage are other common manifestations of pulmonary involvement in systemic vasculitis. Pleural effusions and inflammatory pleural pseudotumours can also occur.

The discovery that antineutrophil cytoplasmic antibodies (ANCA) are strongly associated with vasculitic disorders and their subsequent characterisation has led to improved understanding of the pathogenic mechanisms underlying vasculitis. This paper will review clinical aspects of pulmonary involvement in the systemic vasculitides and explores the impact which the discovery of auto-antibodies in these disorders has had on our understanding of their pathogenesis. It also investigates how these discoveries can be used to improve the management of patients with these conditions.