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