Shrinking lung syndrome in a 14-year-old boy with systemic lupus erythematosus

Pulmonary complications occur frequently in people with systemic lupus erythematosus. We report on an adolescent with an acute onset of dyspnea and pleuritic chest pain with severe restrictive lung physiology on pulmonary function testing (forced vital capacity, 20% of predicted) who had no evidence of parenchymal lung or pleural disease. He was found to have restricted diaphragmatic movement as assessed by fluoroscopy, without evidence of generalized respiratory muscle weakness. His clinical presentation and results of diagnostic tests were typical for shrinking lung syndrome. Given the rarity of shrinking lung syndrome in the pediatric age range, many clinicians are not aware of it as a clinical entity. Shrinking lung syndrome should be included in the differential diagnosis of dyspnea in both children and adults with systemic lupus erythematosus.