Abstract: Objective　To investigate the clinical characteristics and prognosis of systemic lupus erythematosus (SLE) with lupus lung injury at the first onset in children. Method　The clinical data of SLE in 159 children from January 2011 to October 2016 were retrospectively analyzed. According to chest imaging, the patients were divided into lupus lung injury group and non-lupus lung injury group. The clinical characteristics and prognosis of the two groups were compared. Results　There were 89 cases (56.0%) in lupus lung injury group, of which only 37 had respiratory symptoms and 10 had pulmonary signs. Chest imaging showed mainly pleural effusion/pleurisy and lung parenchymal lesions. SLE disease activity index score (SLEDAI) and the proportion of autoimmune hemolytic anemia, hematuria, proteinuria and low complement C3 in lupus lung injury group were higher than those in non-lupus lung injury group, and the difference was statistically significant (P<0.05). All 14 death cases were from lupus lung injury group. The mortality rate of lupus pulmonary injury group was significantly higher than that of non-lupus pulmonary injury group (P<0.05). Conclusion　SLE is prone to have lupus lung injury at the first onset in children, but there are no specific manifestations. Chest imaging is very important. SLE with lupus lung injury at the first onset in children has poor prognosis.

Key words: 　systemic lupus erythematosus;　lupus lung injury;　clinical characteristic;　prognosis;　child