Children with rheumatic disease have rare pulmonary manifestations that may cause significant morbidity and mortality. These children are often clinically asymptomatic until disease has significantly progressed, so they should be screened for pulmonary involvement. There has been recent recognition of a high mortality-related lung disease in systemic-onset juvenile idiopathic arthritis; risk factors include onset of juvenile idiopathic arthritis less than 2 years of age, history of macrophage activation syndrome, presence of trisomy 21, and history of anaphylactic reaction to biologic therapy. Early recognition and treatment of lung disease in children with rheumatic diseases may improve outcomes.