JSLE is an autoimmune disease, characterized by the presence of a wide variety of autoantibodies resulting in multisystem inflammation and organ damage. Compared to younger age groups, the postpubertal age group has a significant female dominance. It presents between 15-20% of individuals before the age of 18 with pulmonary involvement occurring in up to 40% of individuals within a year of diagnosis, doubling at any point during the disease process. In pediatric patients with JSLE, ILD is rare, but it may occur as a complication of acute lupus pneumonitis. It may also develop more insidiously with a chronic non-productive cough, breathlessness on exertion and pleuritic chest pain. The presence of anti-Sm and anti-RNP antibodies may increase the risk of ILD, although this remains controversial.