Current modalities in the diagnosis of pulmonary vasculitis.

Introduction: This review addresses the pulmonary manifestations of the vasculitides, with a focus on diagnostic modalities. Haemorrhagic presentations (usually associated with nephritis: the pulmonary-renal syndrome) are the most common vasculitic cause of early death.

Areas covered: The diagnostic modalities in the pulmonary vasculitides are reviewed, with a focus on primary systemic vasculitis. A literature search of original research and review articles on pulmonary vasculitides was undertaken using the PubMed database.

Expert opinion: Small-vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, especially granulomatosis with polyangiitis (Wegener's granulomatosis) are the most frequent causes of pulmonary vasculitis and typically present as nodules, alveolar infiltrates (haemorrhagic or not), cavities or tracheobronchial stenosis. Lung involvement is less common in large-vessel vasculitis when pulmonary vascular abnormalities can be seen. No single test is pathogonomonic and diagnosis requires integration of clinical, laboratory, imaging and histological findings. Treatment follows similar regimens to other vasculitic presentations, with glucocorticoids in conjunction with immunosuppressive agents, and management of intercurrent sepsis and the increased risk of cardiovascular and thromboembolic events. Prompt diagnosis and intensive treatment of pulmonary vasculitis is essential to improve early mortality and long-term outcomes.