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

CONTEXT: 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.

OBJECTIVES: To identify and characterize the disease process and management of interstitial lung disease related to systemic juvenile idiopathic arthritis.

STUDY DESIGN: 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.

DATA EXTRACTION: All data was extracted through electronic medical records and individually reviewed by two pediatric pulmonologists and two pediatric rheumatologists.

RESULTS: 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.

LIMITATIONS: The cohort size was small and data is reflective of one center's approach to management of a rare lung disease process.

CONCLUSION: 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.