ObjectiveTo investigate the clinical features,treatment and pathogenesis of shrinking lung syndrome (SLS). MethodsA patient of systemic lupus erythematosus(SLE)with SLS as initial manifestation was reported.Literatures about SLS were comprehensively reviewed for its clinical characteristics and pathogenesis. ResultsA 27-year-old man was admitted with chief complaint of intermittent dyspnea and chest pain in 2009.Chest X-ray showed small lung volume and elevated diaphragms.Chest CT revealed bilateral pleural thickness and multiple atelectasis,without significant sign of interstitial fibrosis.Lung function showed restrictive pattern.Routine blood test revealed declined white blood cell and platelet count.C3 and C4 were declined.The autoantibody test revealed positive ANA,dsDNA,AHA,and RNP antibody.A diagnosis of SLE with SLS was made.The patient received corticosteroids and immunosuppressive therapy and was followed up for 5 years.His symptoms had relieved,but there was no obvious improvement in chest radiology and lung function.A total of 118 SLS cases were recorded in literature review,among which 100 were females and 18 were males.The average age was 32.98±14.66 years.Common clinical features of SLS included dyspnea,chest pain,orthopnea,breathlessness,and decreased breath sound.Radiology revealed small lung volume,elevated diaphragm,and dysfunction of diaphragm.Lung function showed restrictive pattern.The primary treatment of the disease was corticosteroid and immunosuppressive agents.The use of theophylline,β-agonist and rituximab was also reported. ConclusionWe should be aware of SLS in patients with SLE or other autoimmune diseases when they present with unexplained dyspnea.