37 - The lungs in rheumatic disease

Abstract

Lung manifestations of rheumatologic diseases can involve all components of the respiratory system, including interstitial lung disease (ILD), airway disease, pleural disease, and vascular diseases. Interstitial lung disease is one of the most challenging lung complications with significant morbidity and mortality. The rheumatologic diseases most often associated with lung disease are scleroderma, rheumatoid arthritis, dermatomyositis/polymyositis, mixed connective tissue disease, and, less frequently, systemic lupus erythematosus and Sjögren syndrome. Some patients may have ILD with features of an autoimmune disease without meeting criteria for a specific connective tissue disease, which has been termed interstitial pneumonia with autoimmune features (IPAF). In addition, pneumonitis can be caused by drugs or opportunistic infection. To date, treatment approaches target the dominant pattern of disease. Given the great challenges that still exist in our understanding of the natural history and treatment of ILD and other pulmonary manifestations in the rheumatic diseases, a multidisciplinary approach among rheumatologists, pulmonologists, radiologists, and pathologists can be of great benefit to this most challenging group of patients.