Department of Pediatric Rheumatology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, No. 109, Xueyuan Road, Wenzhou, 325027, China.
Department of Ultrasound Imaging, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, China.
Pediatr Rheumatol Online J. 2024 Oct 24;22(1):96. doi: 10.1186/s12969-024-01028-5.
Interstitial lung disease (ILD) is a serious complication in systemic juvenile idiopathic arthritis (SJIA). This study aimed to identify the clinical characteristics and prognosis of SJIA-ILD.
A two-center retrospective cohort study was conducted on patients newly diagnosed with SJIA in China from October 2010 to December 2021. Clinical characteristics, laboratory parameters, outcomes, and relapse rates were compared between ILD and non-ILD groups.
A total of 176 children with SJIA were included, including 35 in ILD group and 141 in non-ILD group. The median age at onset of SJIA was 5.8 years (range 4.4-9.5) in patients with SJIA-ILD. It exhibited higher incidences of cervical spine (28.6%) and hip involvement (40.0%) in ILD group (P = 0.031 and P = 0.029, respectively). The incidence of macrophage activation syndrome (MAS) in ILD group reached up to 40%, significantly elevated than that in non-ILD group (P = 0.047). Children with ILD demonstrated a stronger inflammatory response and were more prone to developing lymphopenia (P = 0.009), requiring more combination therapy (P = 0.006) to control disease activity. 54.3% of patients received biologic therapies, with only three patient receiving biologics (one with IL-6 blockade, two with TNF inhibitor) prior to ILD onset and none receiving IL-1 blockade. The median follow-up duration was 6.0 years (range 3.9-9.5). The proportions of patients with SJIA-ILD achieving clinical inactive disease without glucocorticoids within 6 to 12 months of the treatment were significantly lower than control group (45.7% vs. 70.2%, P = 0.006). In ILD group, only 54.3% of patients achieved complete remission, and 17.1% were in a non-remission state, among whom two deaths from respiratory failure. There was no significant difference in disease relapse rates between the two groups (P > 0.05).
Patients with SJIA-ILD exhibited heightened inflammation, increased hip joint and cervical spine involvement, and were more susceptible to developing lymphopenia and MAS, suggesting a relatively poor prognosis. They required a prolonged time to control inflammation and more aggressive treatment strategies to achieve inactive status. The unsatisfactory rate of complete remission highlighted an urgent need for focused clinical strategies.
间质性肺病（ILD）是全身性幼年特发性关节炎（SJIA）的严重并发症。本研究旨在确定 SJIA-ILD 的临床特征和预后。
对 2010 年 10 月至 2021 年 12 月期间在中国新诊断为 SJIA 的患者进行了一项两中心回顾性队列研究。比较了 ILD 组和非 ILD 组之间的临床特征、实验室参数、结局和复发率。
共纳入 176 例 SJIA 患儿，ILD 组 35 例，非 ILD 组 141 例。SJIA-ILD 患儿的 SJIA 发病中位年龄为 5.8 岁（范围 4.4-9.5）。ILD 组的颈椎（28.6%）和髋关节受累（40.0%）发生率较高（P=0.031 和 P=0.029）。ILD 组 MAS 发生率高达 40%，明显高于非 ILD 组（P=0.047）。ILD 组患儿炎症反应更强，更易发生淋巴细胞减少（P=0.009），需要更多联合治疗（P=0.006）来控制疾病活动度。54.3%的患者接受了生物治疗，只有 3 例患者在ILD 发病前接受了生物治疗（1 例 IL-6 阻断剂，2 例 TNF 抑制剂），没有患者接受 IL-1 阻断剂。中位随访时间为 6.0 年（范围 3.9-9.5）。ILD 组在治疗后 6-12 个月内达到临床无激素缓解的患者比例明显低于对照组（45.7%比 70.2%，P=0.006）。在 ILD 组中，只有 54.3%的患者达到完全缓解，17.1%的患者处于非缓解状态，其中 2 例因呼吸衰竭死亡。两组疾病复发率无统计学差异（P>0.05）。
SJIA-ILD 患儿炎症反应更强烈，髋关节和颈椎受累增加，更易发生淋巴细胞减少和 MAS，提示预后相对较差。他们需要更长的时间来控制炎症，需要更积极的治疗策略才能达到无活动状态。完全缓解的不理想率突显了迫切需要有针对性的临床策略。
