Pulmonary disease in systemic lupus erythematosus

Pulmonary problems are common in systemic lupus erythematosus, and may be the presenting feature of this multi-system disease. The clinical spectrum ranges from mild, self-limited, pleuritic chest pain to fulminant and rapidly fatal, diffuse, pulmonary hemorrhage. Accordingly, treatment must be individually tailored to the clinical features of each patient. Non-steroidal-anti-inflammatory drugs may be adequate therapy for pleuritic pain. High dose corticosteroids may be indicated in more severe cases of pleurisy with effusion, lupus pneumonitis, and diffuse interstitial lung disease. Immunosuppressive drugs such as azathioprine and cyclophosphamide should be considered in cases of lupus pneumonitis or interstitial lung disease unresponsive to steroids. Combined therapy with corticosteroids, immunosuppressives and plasmapheresis should be considered for fulminant cases of diffuse pulmonary hemorrhage attributed to lupus. There is no definitive therapy for pulmonary hypertension at this time. Decisions regarding treatment in each instance must be made with the recognition that there is little strong clinical evidence to support the use of any of these therapies. Finally, no pulmonary process should be attributed to lupus until infection has been rigorously excluded in these patients.