High-Mortality Lung Disease Increased in Systemic JIA Patients

Investigators from Cincinnati Children’s Hospital Medical Center (CCHMC), Cincinnati, OH, and Stanford University, Stanford, CA, conducted a prospective study to describe the clinical, immunologic, and pathologic characteristics in children with systemic juvenile idiopathic arthritis (SJIA) who develop lung disease (LD) and to identify risk factors for development of LD in SJIA patients. Study participants were children with SJIA followed at CCHMC who developed LD or patients with SJIA-LD who were referred to CCHMC since 2010. The diagnosis of SJIA in study children was confirmed using specific guidelines, and the presence of LD was determined based on objective findings on physical examination and/or imaging studies. Clinical data, including interleukin-18 (IL-18) levels, imaging findings, development of macrophage activation syndrome (MAS), and history of adverse response …