Pediatric systemic juvenile idiopathic arthritis-related lung disease: Description of clinical cohort and review of management.

Abstract

Since the early 2010s, there has been an increased awareness of interstitial lung disease in systemic juvenile idiopathic arthritis (sJIA-LD) in pediatric patients. Despite the increase in prevalence of sJIA-LD, little is known about this disease process and effective therapeutic management. To identify and characterize the disease process and management of interstitial lung disease related to systemic juvenile idiopathic arthritis. 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 sJIA-LD. All data was extracted through electronic medical records and individually reviewed by two pediatric pulmonologists and two pediatric rheumatologists. 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 peripheral lymphadenopathy and hepatosplenomegaly. The cohort size was small and data is reflective of one center's approach to management of a rare lung disease process. Interstitial lung disease due to sJIA is rare and management can be difficult in these complex patients.More research is necessary to understand the increased incidence and treatment of sJIA-LD in pediatric population.