Pediatric Systemic Juvenile Idiopathic Arthritis related Lung Disease: Description of clinical cohort and review of management.

Beginning in the early 2010s, an increased incidence of interstitial lung disease in systemic juvenile idiopathic arthritis (sJIA-LD) in pediatric patients has been identified. Despite the increase in prevalence of sJIA-LD, little is known about this disease process and effective therapeutic management. In this single-center, retrospective case series of 9 patients, we analyze demographic, clinical, radiographic, and laboratory data to corroborate common clinical characteristics and describe an approach for diagnosis and monitoring of interstitial lung disease in children with sJIA. Our results were similar to other described cases of sJIA-LD as patients in our cohort were more likely to be younger, have a history of macrophage activation syndrome and prior use of biologic therapies. In contrast to prior studies, they did not present with lymphadenopathy and hepatosplenomegaly. We discuss our management of this rare disease process. More research is necessary to understand the increased incidence and treatment of sJIA-LD in pediatric population.