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1.重庆医科大学附属儿童医院超声科,儿童少年健康与疾病国家临床医学研究中心,儿童发育疾病研究教育部重点实验室,儿童代谢与炎症性疾病重庆市重点实验室,重庆
3.重庆佑佑宝贝妇儿医院超声科,重庆
2.重庆医科大学附属儿童医院风湿免疫科,重庆
肖欢,E-mail: huan106689210@163.com
收稿:2026-06-02,
修回:2026-08-04,
录用:2026-08-06,
纸质出版:2026-09-30
移动端阅览
牟芳婷,邱敏,尹春梅,等.幼年特发性关节炎不同亚型的超声表型特征及其疾病活动度评估的差异化策略探索:一项横断面研究[J].陆军军医大学学报,2026,48(18):2633-2644.DOI:10.16016/j.2097-0927.202606012.
Mou F T,Qiu M,Yin C M,et al.Ultrasonographic phenotypes of different juvenile idiopathic arthritis subtypes and differentiated strategies for disease activity assessment: a cross-sectional study[J].J Army Med Univ,2026,48(18):2633-2644.DOI:10.16016/j.2097-0927.202606012.
牟芳婷,邱敏,尹春梅,等.幼年特发性关节炎不同亚型的超声表型特征及其疾病活动度评估的差异化策略探索:一项横断面研究[J].陆军军医大学学报,2026,48(18):2633-2644.DOI:10.16016/j.2097-0927.202606012. DOI:
Mou F T,Qiu M,Yin C M,et al.Ultrasonographic phenotypes of different juvenile idiopathic arthritis subtypes and differentiated strategies for disease activity assessment: a cross-sectional study[J].J Army Med Univ,2026,48(18):2633-2644.DOI:10.16016/j.2097-0927.202606012. DOI:
目的
2
幼年特发性关节炎(juvenile idiopathic arthritis,JIA)各亚型异质性显著,传统评估对亚临床滑膜炎识别不足。肌肉骨骼超声可敏感显示滑膜增厚、关节积液及异常血流信号,但不同亚型的超声表型特征尚缺乏系统比较。本研究旨在明确JIA不同亚型的特征性关节受累模式,为JIA的个体化超声评估提供依据。
方法
2
采用横断面研究设计方案,纳入重庆医科大学附属儿童医院2023年11月至2024年10月收治的261例JIA患儿(女135例,占51.7%;年龄中位数9.0岁,病程中位数为21.5个月),涵盖多关节型(pJIA,77例)、全身型(sJIA,67例)、少关节型(oJIA,48例)、附着点炎相关关节炎(ERA,36例)及未分类型(uJIA,33例)5种亚型。按标准化方案对68个外周关节行超声检查,依据国际风湿病结局评价定义及儿童关节炎与风湿病研究联盟半定量评分体系评估灰阶(grayscale,GS)及能量多普勒(power doppler,PD)超声信号。由2名对临床资料设盲的肌骨超声医师独立完成检查与评分;另从40例患儿中随机抽取GS、PD静态图像各75幅,由2名医师独立评分以评价观察者间一致性。疾病活动度采用JADAS27评分评估(活动期JADAS27
>
1)。在主体横断面研究中,以亚型及超声评分为分组/预测变量,以关节超声阳性率、GS/PD评分及JADAS27定义的疾病活动状态为结局指标。采用层次聚类分析受累模式,
χ
2
检验筛选亚型间差异关节,Spearman相关分析评价超声评分与JADAS27的相关性,ROC曲线评价诊断效能。设置一个小样本(
n
=37)纵向随访评价治疗前后变化。
结果
2
①评分体系观察者间一致性优:GS评分ICC=0.87(95%
CI
:0.78~0.95),PD评分ICC=0.85(95%
CI
:0.78~0.91)。共评估17 748个关节,检出滑膜炎829个(4.7%),PD阳性525个(3.0%)。②各亚型呈可区分受累模式:多关节型(polyarticular JIA,pJIA)广泛关节受累[(6.2±10.0)个/例],手部小关节突出(近端指间32.5%、掌指19.5%);全身型(sJIA)以大关节为主[(3.1±4.3个/例)],肩关节受累率19.4%,阳性者81.3%为sJIA;少关节型(oJIA)受累最少[(0.9±1.4个/例)],以膝为主(18.8%);附着点炎相关关节炎(enthesitis-related arthritis,ERA)为(1.5±1.3)个/例。肩、肘、腕、掌指、近端指间、远端指间关节亚型间差异有统计学意义(校正
P
<
0.05)。手-腕关节特征提示pJIA的AUC为0.61;肩关节阳性提示sJI
A特异度达0.99。③PD评分与JADAS27相关性(
r
=0.54,
P
<
0.001)强于GS评分(
r
=0.48,
P
<
0.001),pJIA中相关系数最高(
r
=0.71,
P
<
0.001)。PD评分对活动期判别效能中等(AUC=0.67,95%
CI
:0.61~0.75,
P
<
0.001),最佳截断值≥1.0,敏感度51%、特异度82%;pJIA中AUC=0.73,sJIA与oJIA均为0.71,ERA中无统计学意义(
P
=0.33)。④纵向随访PD可反映治疗效果:平均4.4个月的纵向随访中,32例活动期患儿的JADAS27评分由8.0(4.0,15.3)分降至3.5(1.0,8.1)分(
P
<
0.001,SRM=-0.74);PD评分由1.0(0,3.0)分降至0(0,1.0)分(
P
=0.003,SRM=-0.25),GS评分变化无统计学意义(
P
=0.67)。
结论
2
JIA不同亚型呈现可区分的超声表型特征:肩关节阳性高度指向sJIA,腕/MCP/PIP阳性倾向pJIA,oJIA与ERA几乎不累及肩及手部小关节。PD评分可敏感反映疾病活动度与治疗反应,但其诊断效能存在亚型差异:在pJIA中与疾病活动度相关性最强,而在sJIA与ERA中相对较弱,需结合全身炎症指标综合判断。
Objective
2
Juvenile idiopathic arthritis (JIA) encompasses highly heterogeneous subtypes
and conventional clinical assessment often fails to detect subclinical synovitis. Musculoskeletal ultrasound can sensitively depict synovial hypertrophy
joint effusion and abnormal blood flow signals
yet the ultrasound phenotypes of different subtypes have not been systematically compared. This study aims to clarify the characteristic joint involvement patterns of different JIA subtypes
providing evidence for individualized ultrasonographic assessment in JIA.
Methods
2
A cross-sectional study was conducted on 261 children with JIA (135 girls
51.7%; median age 9.0 years; median disease duration 21.5 months) admitted to the Children's Hospital of Chongqing Medical University between November 2023 and October 2024
encompassing 5 subtypes: polyarticular JIA (pJIA
n
=77)
systemic JIA (sJIA
n
=67)
oligoarticular JIA (oJIA
n
=48)
enthesitis-related arthritis (ERA
n
=36)
and undifferentiated JIA (uJIA
n
=33). Ultrasonography was performed on
68 peripheral joints according to a standardized protocol. Grayscale (GS) synovitis and power Doppler (PD) signals were assessed using the Outcome Measures in Rheumatology definitions and the semiquantitative scoring system proposed by the Childhood Arthritis and Rheumatology Research Alliance. Examinations and scoring were independently completed by 2 musculoskeletal ultrasound physicians blinded to clinical data. Additionally
75 GS and 75 PD static images were randomly selected from 40 children for independent scoring by 2 physicians to evaluate interobserver agreement. Disease activity was evaluated using the JADAS27 score (active disease defined as JADAS27
>
1). In the main cross-sectional analysis
JIA subtype and ultrasound scores served as grouping/predictor variables
while joint-level ultrasound positivity rate
GS/PD scores
and disease activity state defined by JADAS27 served as outcome measures. Hierarchical clustering analysis was carried out to analyze involvement patterns
Chi
-square test to identify joints with significant differences across subtypes
Spearman correlation analysis to evaluate the correlations between ultrasound scores and JADAS27
and ROC curve analysis to assess diagnostic performance. A small-sample longitudinal follow-up subset (square test to identify joints with significant differences across subtypes
Spearman correlation analysis to evaluate the correlations between ultrasound scores and JADAS27
and ROC curve analysis to assess diagnostic performance. A small-sample longitudinal follow
n
=37) was included to evaluate the changes before and after treatment.
Results
2
①The interobserver agreement of the scoring system was excellent
with intraclass correlation coefficients of 0.87 (95%
CI
: 0.78 to 0.95) for GS scores and 0.85 (95%
CI
: 0.78 to 0.91) for PD scores. A total of 17 748 joints were evaluated
with synovitis detected in 829 joints (4.7%) and PD positivity in 525 joints (
3.0%). ②Distinct patterns of joint involvement were observed across subtypes: pJIA showed extensive polyarticular involvement (6.2±10.0 joints per case)
with prominent small-joint involvement in the hands (proximal interphalangeal 32.5%
metacarpophalangeal 19.5%); sJIA predominantly involved large joints (3.1±4.3 joints per case)
with shoulder involvement in 19.4% of patients (81.3% of those with shoulder involvement had sJIA); oJIA had the fewest involved joints (0.9±1.4 per case)
predominantly the knee (18.8%); ERA had a mean of 1.5±1.3 involved joints per case. Significant differences across subtypes were found in the shoulder
elbow
wrist
metacarpophalangeal
proximal interphalangeal
and distal interphalangeal joint (adjusted
P
<
0.05). The hand-wrist joint feature (positivity in wrist/MCP/PIP) yielded an AUC value of 0.61 for identifying pJIA; shoulder joint positivity showed a specificity of 0.99 for sJIA. PD scores were correlated more strongly with JADAS27 score (
r
=0.54
P
<
0.001) than GS scores (
r
=0.48
P
<
0.001)
with the highest correlation coefficient observed in pJIA (
r
=0.71
P
<
0.001). ③PD scores demonstrated moderate diagnostic performance for discriminating active disease (AUC=0.67
95%
CI
: 0.61 to 0.75
P
<
0.001)
with an optimal cutoff of ≥1.0
a sensitivity of 51% and a specificity of 82%; the AUC value was 0.73 in pJIA
0.71 in both sJIA and oJIA
and not statistically significant in ERA (
P
=0.33). ④Longitudinal follow-up showed that PD scores could reflect treatment response. During a mean follow-up of 4.4 months in 32 patients with active disease at baseline
the JADAS27 score was decreased from 8.0 (4.0
15.3) to 3.5 (1.0
8.1) (
P
<
0.001
SRM=-0.74); PD scores were decreased from 1.0 (0
3.0) to 0 (0
1.0) (
P
=0.003
SRM=-0.25)
whereas GS scores showed no significant change
(
P
=0.67).
Conclusion
2
Distinct ultrasonographic phenotypes are identified across JIA subtypes: shoulder positivity strongly points to sJIA
wrist/MCP/PIP positivity tends to pJIA
while oJIA and ERA rarely involve the shoulder or small joints of the hands. The PD score can sensitively reflect disease activity and treatment response
but its diagnostic performance varies among subtypes: strongest correlation with disease activity in pJIA
whereas relatively weak in sJIA and ERA
requiring comprehensive assessment in considering systemic inflammatory indicators.
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