Pulmonary manifestations of systemic vasculitis

Primary systemic vasculitides are associated with a wide variety of pulmonary manifestations, including vascular features (aneurysm, thrombosis, pulmonary hypertension, alveolar hemorrhage), parenchymal involvement (infiltrates, interstitial lung disease, nodular changes, emphysema), airway stenoses, lymphadenopathy, and pleural effusions. Small-, medium-, and large-vessel vasculitides have variable patterns of pulmonary involvement that are important to identify and differentiate. The recognition of the pulmonary manifestations in the context of the vasculitis subtype and clinical phenotype can guide the appropriate diagnostic evaluation. Imaging modalities, the direct visualization of airways, and pulmonary function testing can be helpful depending on the type of pulmonary involvement suspected. Clinicians should maintain an appropriate degree of suspicion for vasculitis in patients presenting with pulmonary symptoms, as well as recognize pulmonary involvement in patients with established vasculitis. The identification of pulmonary involvement through directed diagnostic evaluation can influence patient monitoring, management, and prognosis. Interdisciplinary management between rheumatologists and pulmonologists can improve the care of patients with systemic vasculitis.