Abstract:  Objectives To analyze the clinical manifestation of pulmonary injury in children with systemic lupus erythematosus (SLE). Methods The clinical data of 8 SLE children with onset of respiratory symptoms as the first sign were retrospectively analyzed from January 2011 to December 2015. Results In these 8 children (4 females and 4 males) aged 6 - 15 years old. All of them presented cough, and 7 cases had fever, 5 cases had anhelation, 3 cases had hemoptysis, 3 cases had stethalgia, 3 cases had dyspnea and 3 cases had cyanosis. The types of pulmonary injury were pleural effusion in 5 cases (62.5%), acute lupus pneumonitis in 4 cases (50.0%), chronic interstitial pneumonia in 2 cases (25.0%), and pneumorrhagia in 2 cases (25.0%). In 7 children who performed chest high resolution CT examination, it showed that 5 cases had ground-glass shadow, 5 cases had pleural effusion, 5 cases had enlargement of mediastinum or lymph nodes, and 4 cases had segmental pulmonary consolidation. After treatment of the primary disease, the respiratory symptoms and pulmonary images were improved rapidly in 7 cases, and one case died of pneumorrhagia. Conclusion The clinical ministrations in SLE children with onset of respiratory symptoms as the first sign were not specific, and it was usually confused with respiratory infection. However, it usually had other organs involved and the chest imaging was more commonly showed groundglass shadow, segmental pulmonary consolidation, and pleural effusion.