University of Pittsburgh, Pittsburg, PA, USA.
Division of Rheumatology, Cincinnati Children's Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, USA.
Pediatr Rheumatol Online J. 2020 Jul 15;18(Suppl 1):53. doi: 10.1186/s12969-020-00444-7.
For reasons poorly understood, and despite the availability of biological medications blocking IL-1 and IL-6 that have markedly improved overall disease control, children with Systemic Juvenile Idiopathic Arthritis (SJIA) are now increasingly diagnosed with life-threatening chronic complications, including hepatitis and lung disease (SJIA-LD). On October 3-4, 2019, a two-day meeting, NextGen Therapies for Systemic Juvenile Idiopathic Arthritis (SJIA) & macrophage activation syndrome (MAS) organized by the Systemic JIA Foundation ( www.systemicjia.org/ ) in Washington, DC brought together scientists, clinicians, parents and FDA representatives with the objectives (1) to integrate clinical and research findings in MAS and SJIA-LD, and (2) to develop a shared understanding of this seemingly new pulmonary complication of SJIA. The current manuscript summarizes discussions and conclusions of the meeting.
由于原因尚不清楚，尽管有阻断白介素-1 和白介素-6 的生物药物可显著改善整体疾病控制，但患有全身型幼年特发性关节炎 (SJIA) 的儿童现在越来越多地被诊断出患有危及生命的慢性并发症，包括肝炎和肺病 (SJIA-LD)。2019 年 10 月 3 日至 4 日，为期两天的会议“下一代治疗全身型幼年特发性关节炎 (SJIA) 和巨噬细胞活化综合征 (MAS)”由华盛顿特区的全身型 JIA 基金会 (www.systemicjia.org/) 组织，汇集了科学家、临床医生、家长和 FDA 代表，目标是 (1) 整合 MAS 和 SJIA-LD 的临床和研究结果，(2) 对 SJIA 的这种看似新的肺部并发症达成共识。本手稿总结了会议的讨论和结论。
