The respiratory system is affected more commonly in systemic lupus erythematosus (SLE) than in any other collagen vascular disease. In this article the many different manifestations of SLE in the pleura, lung parenchyma, lung vasculature, upper and lower airways, and respiratory muscles are reviewed: Moreover, indirect involvement of the respiratory system with SLE is discussed with special reference to pulmonary infection and drug-induced SLE pulmonary disease.