Abstract
BACKGROUND: Neonatal lupus is a rare autoimmune condition caused by the transplacental passage of maternal autoantibodies, most commonly from mothers with systemic lupus erythematosus. While cutaneous and cardiac involvement are well recognized, respiratory manifestations-particularly acute lupus pneumonitis-are extremely rare, with very few documented cases in neonates. CASE PRESENTATION: We describe a preterm neonate of Middle Eastern ethnicity born at 34 weeks gestation to a mother with systemic lupus erythematosus who presented with erythematous skin lesions and progressive respiratory distress. The initial suspicion was neonatal sepsis, but further imaging revealed bilateral ground-glass opacities suggestive of pneumonitis. The diagnosis of acute lupus pneumonitis was considered. The patient was treated with intravenous immunoglobulin and corticosteroids, resulting in significant clinical improvement. CONCLUSION: This case highlights the importance of considering acute lupus pneumonitis in neonates with unexplained respiratory failure, especially when maternal autoimmune disease is present. Early recognition and immunosuppressive therapy can lead to favorable outcomes.