Background: Neonatal lupus erythematosus (NLE) is an uncommon immunemediated disease caused by the transplacental passage of maternal autoantibodies. In this paper, we described a case of a newborn with NLE involving lung damage from clinical diagnoses to autopsy findings. Case presentation: The mother had no history of immune system disease; however, the test for antinuclear antibodies was positive. A boy was delivered spontaneously at 36+2 weeks' gestation without asphyxia (birth weight: 1.21

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... kg, length: 38 cm, head circumference: 26 cm). Soon after birth, intermittent shortness of breath occurred, followed by cyanosis and severe hypoxemia. Blood tests suggested that the infant was positive for anti-nuclear antibodies. Although multiple respiratory support methods were used, death could not be avoided and occurred at 6.5 days due to cardiopulmonary failure. The results of the autopsy revealed diffuse interstitial inflammation and interstitial fibrosis in both lungs, manifesting as telangiectasia, hyperemia, alveolar cavity dilatation and fusion, and obvious interstitial widening with chronic inflammatory cell infiltration. The newborn was finally diagnosed with nonspecific interstitial pneumonia with fibrosis caused by congenital systemic lupus erythematosus. Conclusions: Pulmonary involvement with NLE is a diagnostic challenge at present. Close attention should be given to newborns whose mothers have systemic lupus erythematosus.