Sjögren syndrome is a chronic inflammatory disorder characterized by exocrine gland dysfunction leading to the sicca complex of dry eyes and mouth. In the primary form, Sjögren syndrome is not associated with another connective tissue disease such as rheumatoid arthritis. A wide spectrum of extraglandular manifestations can be seen in patients with Sjögren syndrome. Pulmonary involvement in Sjögren syndrome includes interstitial lung disease, airway dryness (xerotrachea), bronchiectasis, bronchiolitis, pleural disease (effusion or pleuritis), and pulmonary vascular disease (pulmonary vasculitis, pulmonary hypertension). In addition, Sjögren syndrome is associated with an increased incidence of lymphoma.