Chronic lung disease in children with systemic juvenile idiopathic arthritis (SJIA-LD) is an emerging and potentially life-threatening disease complication. Despite recent descriptions of its clinical spectrum, preliminary immunologic characterization, and proposed hypotheses regaarding etiology, optimal approaches to diagnosis and management remain unclear. Here, we review the current clinical understanding of SJIA-LD, including the potential role of biologic therapy in disease pathogenesis, as well as the possibility of drug reactions with eosinophilia and systemic symptoms (DRESS). We discuss approaches to evaluation of children with suspected SJIA-LD, including a proposed algorithm to risk-stratify all SJIA patients for screening to detect LD early. We review potential pharmacologic and non-pharmacologic treatment approaches that have been reported for SJIA-LD or utilized in interstitial lung diseases associated with other rheumatic diseases. This includes lymphocyte-targeting therapies, JAK inhibitors, and emerging therapies against IL-18 and IFNγ. Finally, we consider urgent unmet needs in this area including in basic discovery of disease mechanisms and clinical research to improve disease detection and patient outcomes.

患有系统性幼年特发性关节炎（SJIA-LD）的儿童的慢性肺病是一种新出现的、可能危及生命的疾病并发症。尽管最近对其临床谱、初步免疫学特征进行了描述，并提出了有关病因学的假设，但诊断和治疗的最佳方法仍不清楚。在这里，我们回顾了目前对 SJIA-LD 的临床认识，包括生物治疗在疾病发病机制中的潜在作用，以及嗜酸性粒细胞增多和全身症状 (DRESS) 药物反应的可能性。我们讨论了评估疑似 SJIA-LD 儿童的方法，包括对所有 SJIA 患者进行风险分层的拟议算法，以便进行早期筛查以早期发现 LD。我们回顾了已报道的 SJIA-LD 或用于与其他风湿性疾病相关的间质性肺疾病的潜在药物和非药物治疗方法。这包括淋巴细胞靶向疗法、JAK 抑制剂以及针对 IL-18 和 IFNγ 的新兴疗法。最后，我们考虑该领域未满足的迫切需求，包括疾病机制的基本发现和改善疾病检测和患者治疗结果的临床研究。