The shrinking lungs syndrome in systemic lupus erythematosus

A comprehensive review of the literature on shrinking lungs syndrome (SLS) in systemic lupus erythematosus involved a MEDLINE search (1965-1997) of ca se reports and clinical series of patients with the diagnosis of SLS, A tot al of 49 well-documented cases of SLS were reviewed. Shrinking Lungs syndro me is characterized by unexplained dyspnea, a restrictive pattern on pulmon ary function test results, and an elevated hemidiaphragm. The cause of SLS remains controversial, with several authors attributing the disorder to dia phragmatic weakness and others suggesting that chest wall restriction accou nts for the clinical syndrome. No definitive therapy exists. Corticosteroid s have been reported to lessen symptoms and improve pulmonary function in s ome patients with SLS, but other methods of treatment have occasionally bee n found to be helpful. Clinical presentation, method of diagnosis, pathogen esis, and treatment modalities are summarized in this review. An uncommon c omplication of systemic lupus erythematosus, SLS causes significant morbidi ty and, occasionally, mortality.