Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study.

IF 2.8 3区 医学 Q1 PEDIATRICS Pediatric Rheumatology Pub Date : 2024-10-24 DOI:10.1186/s12969-024-01028-5
Wenting Zhan, Jinxiang Yang, Lingzhi Qiu, Kangkang Yang, Xiaohua Ye, Yaoyao Shangguan, Haiguo Yu, Wenjie Zheng
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Abstract

Background: 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.

Methods: 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.

Results: 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).

Conclusions: 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.

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系统性幼年特发性关节炎间质性肺病的临床特征和预后:一项双中心回顾性队列研究。
背景:间质性肺病(ILD)是系统性幼年特发性关节炎(SJIA)的一种严重并发症。本研究旨在确定SJIA-ILD的临床特征和预后:方法:2010年10月至2021年12月,一项由两个中心进行的回顾性队列研究对中国新诊断的SJIA患者进行了研究。比较了ILD组和非ILD组的临床特征、实验室指标、预后和复发率:结果:共纳入176名SJIA患儿,其中ILD组35名,非ILD组141名。SJIA-ILD患者的中位发病年龄为5.8岁(4.4-9.5岁)。在ILD组中,颈椎(28.6%)和髋关节(40.0%)受累的发病率较高(分别为P = 0.031和P = 0.029)。ILD组的巨噬细胞活化综合征(MAS)发生率高达40%,明显高于非ILD组(P = 0.047)。ILD患儿的炎症反应更强,更容易出现淋巴细胞减少症(P = 0.009),需要更多的联合疗法(P = 0.006)来控制疾病活动。54.3%的患者接受了生物制剂治疗,仅有3名患者在ILD发病前接受了生物制剂治疗(1人使用IL-6阻断剂,2人使用TNF抑制剂),没有人接受IL-1阻断剂治疗。中位随访时间为 6.0 年(3.9-9.5 年不等)。SJIA-ILD患者在治疗后6至12个月内实现临床非活动性疾病而无需使用糖皮质激素的比例明显低于对照组(45.7% vs. 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 人死于呼吸衰竭。两组患者的疾病复发率无明显差异(P>0.05):结论:SJIA-ILD患者的炎症反应加剧,髋关节和颈椎受累加重,更容易出现淋巴细胞减少和MAS,预后相对较差。他们需要更长的时间来控制炎症,采取更积极的治疗策略才能达到非活动状态。完全缓解率并不令人满意,这凸显出迫切需要重点突出的临床策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Pediatric Rheumatology
Pediatric Rheumatology PEDIATRICS-RHEUMATOLOGY
CiteScore
4.10
自引率
8.00%
发文量
95
审稿时长
>12 weeks
期刊介绍: Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects. The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.
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