Department of Rheumatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Clin Exp Rheumatol. 2024 Nov;42(11):2303-2311. doi: 10.55563/clinexprheumatol/udjbtq. Epub 2024 Sep 9.
In recent years, the distinct clinical presentations and elevated mortality rates of various subtypes of juvenile idiopathic arthritis (JIA) with pulmonary involvement have garnered significant attention. This study aimed to elucidate the clinical characteristics of pulmonary involvement in patients with JIA to improve clinicians' knowledge.
This single-centre retrospective study analysed the baseline data, treatment options, follow-up of sixty patients of JIA with pulmonary involvement in China. Patients with interstitial lung disease (ILD) were further classified in accordance with the 2013 American Thoracic Society/European Respiratory Society International multidisciplinary consensus on idiopathic interstitial pneumonia.
Sixty patients (5.03%) with JIA were complicated with pulmonary involvement. The highest subtype was systemic JIA (sJIA, 63.3%), followed by rheumatoid factor (RF)-positive polyarthritis (pJIA, 25.0%). The incidence of macrophage activation syndrome (MAS) was 21.6%. The most common diagnosis was ILD (90%). Respiratory symptoms/signs were initially experienced by 61.7% of the patients, and respiratory support was required by 21.7%. High-resolution CT classification of sJIA revealed non-specific interstitial pneumonia (NSIP) and organising pneumonia. High-resolution CT classification of pJIA was NSIP and usually interstitial pneumonia (UIP). Patients were treated with NSAIDs, along with glucocorticoids, DMARDs, and biological agents. The survival rates after 1 and 5 years were approximately 93.3% and 90.0%, respectively.
Patients with JIA with pulmonary involvement present with early onset, high mortality rate. JIA patients should undergo physical examination thoroughly and high-resolution CT scans, lung function tests for evaluating and monitoring the occurrence and development of pulmonary involvement in early stages to improve prognosis.
近年来，具有肺部受累的各种幼年特发性关节炎（JIA）亚型的明显临床表现和死亡率升高引起了广泛关注。本研究旨在阐明 JIA 患者肺部受累的临床特征，以提高临床医生的认识。
本单中心回顾性研究分析了中国 60 例 JIA 合并肺部受累患者的基线数据、治疗选择和随访情况。ILD 患者根据 2013 年美国胸科学会/欧洲呼吸学会国际特发性间质性肺炎多学科共识进一步分类。
60 例（5.03%）JIA 患者合并肺部受累。最高亚型为全身型 JIA（sJIA，63.3%），其次是类风湿因子阳性多关节炎（pJIA，25.0%）。MAS 的发生率为 21.6%。最常见的诊断是ILD（90%）。61.7%的患者最初出现呼吸道症状/体征，21.7%的患者需要呼吸支持。sJIA 的高分辨率 CT 分类显示非特异性间质性肺炎（NSIP）和机化性肺炎。pJIA 的高分辨率 CT 分类为 NSIP 和通常的间质性肺炎（UIP）。患者接受 NSAIDs 联合糖皮质激素、DMARDs 和生物制剂治疗。1 年和 5 年的生存率分别约为 93.3%和 90.0%。
具有肺部受累的 JIA 患者发病早，死亡率高。JIA 患者应进行彻底的体格检查和高分辨率 CT 扫描、肺功能检查，以评估和监测肺部受累的发生和发展，从而改善预后。
