Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease

作者：Yannan Huang, Laura Sompii‐Montgomery, Jessica Patti, Alex Pickering, Shima Yasin, Thuy Do, Elizabeth Baker, Denny Gao, Rabheh Abdul‐Aziz, Edward M. Behrens, Scott Canna, Matthew Clark, Dominic O. Co, Kathleen Collins, B. Anne Eberhard, Monica Friedman, T. Brent Graham, Timothy Hahn, Aimee O. Hersh, Patricia M. Hobday, Michael J. Holland, Jennifer Huggins, Pai‐Yue Lu, Melissa L. Mannion, Cynthia K. Manos, Jessica Neely, Karen Onel, Amir B. Orandi, Andrea Ramirez, Adam Reinhardt, Mona Riskalla, L E Santiago, Matthew L. Stoll, Tracy V. Ting, Alexei A. Grom, C. Towe, Grant S. Schulert · 发表于：Arthritis Care & Research · 年份：2023 · DOI：10.1002/acr.25234 · 被引用次数：27 · 研究领域：Autoimmune and Inflammatory Disorders Research、Adolescent and Pediatric Healthcare、Pediatric health and respiratory diseases

OBJECTIVE: Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is a life-threatening disease complication. Key questions remain regarding clinical course and optimal treatment approaches. The objectives of the study were to detail management strategies after SJIA-LD detection, characterize overall disease courses, and measure long-term outcomes. METHODS: This was a prospective cohort study. Clinical data were abstracted from the electronic medical record, including current clinical status and changes since diagnosis. Serum biomarkers were determined and correlated with presence of LD. RESULTS: We enrolled 41 patients with SJIA-LD, 85% with at least one episode of macrophage activation syndrome and 41% with adverse reactions to a biologic. Although 93% of patients were alive at last follow-up (median 2.9 years), 37% progressed to requiring chronic oxygen or other ventilator support, and 65% of patients had abnormal overnight oximetry studies, which changed over time. Eighty-four percent of patients carried the HLA-DRB1*15 haplotype, significantly more than patients without LD. Patients with SJIA-LD also showed markedly elevated serum interleukin-18 (IL-18), variable C-X-C motif chemokine ligand 9 (CXCL9), and significantly elevated matrix metalloproteinase 7. Treatment strategies showed variable use of anti-IL-1/6 biologics and addition of other immunomodulatory treatments and lung-directed therapies. We found a broad range of current clinical status indep...