伴关节症状川崎病与川崎病样症状起病全身型幼年特发性关节炎的早期鉴别：一项单中心回顾性研究

doi:10.12372/jcp.2026.26e0076

临床儿科杂志 ›› 2026, Vol. 44 ›› Issue (6): 489-498.doi: 10.12372/jcp.2026.26e0076

伴关节症状川崎病与川崎病样症状起病全身型幼年特发性关节炎的早期鉴别：一项单中心回顾性研究

刘子尧¹^,², 王聪颖², 王宏茂², 张明明², 许瑛杰³, 赖建铭³, 牛文全⁴, 李晓惠¹^,⁵()

¹ 北京大学首都儿科研究所教学医院（北京 100020）
² 首都医科大学附属首都儿童医学中心心血管内科（北京 100020）
³ 首都医科大学附属首都儿童医学中心风湿免疫科（北京 100020）
⁴ 首都儿科研究所循证医学中心（北京 100020）
⁵ 清华大学北京清华长庚医院（北京 102218）

收稿日期:2026-01-23 修回日期:2026-04-01 录用日期:2026-04-16 出版日期:2026-06-15 发布日期:2026-06-04
通讯作者: 李晓惠 E-mail:lxhmaggie@pumc.edu.cn
作者简介:第一联系人：作者贡献（Authors’ Contributions）
刘子尧负责收集、分析数据、撰写论文初稿、具体实施研究，王聪颖、王宏茂、张明明、许瑛杰、赖建铭负责收集、整理数据，牛文全负责分析数据、具体实施研究，李晓惠负责选题、设计研究、论文修改和经费支持。所有作者对要发布的版本给予最终批准，并同意对本论文负责。
基金资助:
国家自然科学基金面上项目(82370511)

Early differentiation of Kawasaki disease with joint symptoms and systemic juvenile idiopathic arthritis with Kawasaki disease-like symptoms: a single-center retrospective study

LIU Ziyao¹^,², WANG Congying², WANG Hongmao², ZHANG Mingming², XU Yingjie³, LAI Jianming³, NIU Wenquan⁴, LI Xiaohui¹^,⁵()

¹ Capital Institute of Pediatrics-Peking University Teaching Hospital, Beijing 100020, China
² Department of Pediatric Cardiology, Capital Center for Children's Health, Capital Medical University, Beijing 100020, China
³ Department of Rheumatology and Immunology, Capital Center for Children's Health, Capital Medical University, Beijing 100020, China
⁴ Center for Evidence-Based Medicine, Capital Institute of Pediatrics, Beijing 100020, China
⁵ Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua Medicine, Tsinghua University, Beijing 102218, China

Received:2026-01-23 Revised:2026-04-01 Accepted:2026-04-16 Published:2026-06-15 Online:2026-06-04
Contact: LI Xiaohui E-mail:lxhmaggie@pumc.edu.cn

摘要/Abstract

摘要：

目的川崎病（KD）患儿可伴有关节症状，部分全身型幼年特发性关节炎（sJIA）患儿以川崎病样症状起病，起病阶段表现与KD相似，该两类患儿的早期鉴别非常困难。本研究旨在分析两类患儿早期临床特征的差异，探索以伴关节表现的KD样症状起病、实际诊断为sJIA患儿的早期预警征象。方法回顾性分析2021年1月至2025年8月于北京大学首都儿科研究所教学医院就诊，早期症状符合伴有关节症状KD诊断的患儿临床资料，所有患儿均完成至少6个月临床随访，根据随访转归分为sJIA组（随访期间最终修正诊断为sJIA）和KD组（随访期间维持KD诊断）。对比两组患儿的发热特征、关节受累模式、炎症指标、冠状动脉病变、治疗反应。结果 71例伴关节症状KD患儿中，男46例、女25例，中位起病年龄4.3（2.5~5.1）岁，中位发热持续时间9.0（7.5~12.5）d，起病至关节症状出现中位时间9.0（6.5~12.0）d。55例（77.5%）为少关节炎，56例（78.9%）为大关节炎，小关节炎仅2例，混合型关节炎13例。冠状动脉瘤发生率为12.7%（9/71），1例患儿随访中发生巨噬细胞活化综合征（MAS）。所有患儿均接受静脉注射免疫球蛋白（IVIg）治疗，32例对初始IVIg治疗无反应。6个月随访期间修正诊断为sJIA患儿（sJIA组）8例、维持KD诊断（KD组）63例。sJIA组中位发热持续时间为24.5 d，显著长于KD组的8.0 d（P<0.01）。起病时CRP水平在sJIA组和KD组之间差异无统计学意义（P>0.05）；首次IVIg治疗后KD组中位CRP水平相较于sJIA组显著降低（12.9 mg/L对61.0 mg/L），ΔCRP及CRP下降率sJIA组均显著低于KD组（P均<0.05）；随访1个月时sJIA组CRP水平显著高于KD组（P<0.001），随访6个月时CRP水平两组间差异无统计学意义（P>0.05）。初诊时，sJIA组中位受累关节数显著多于KD组，sJIA组多关节炎、混合型关节炎比例，腕关节和指间关节受累比例，滑膜增厚发生率高于KD组，差异均有统计学意义（P<0.05）。随访1个月及6个月时，sJIA组受累关节数均显著高于KD组（P均<0.001）。以发热持续时间和受累关节数作为鉴别sJIA组与KD组的指标时，AUC分别为0.89和0.81。所有sJIA患儿（100%，8/8）均对初始IVIg治疗无反应，而KD组IVIg无反应率为38.1%（24/63），差异有统计学意义（P<0.05）。两组冠状动脉病变发生率差异无统计学意义（P>0.05）。结论发热持续时间长、多关节或混合型关节炎、腕关节及指间小关节受累、影像学提示滑膜增厚、关节症状迁延不愈，以及对IVIg治疗无反应，是伴关节症状KD患儿最终修正诊断为sJIA的早期临床预警征。

关键词: 全身型幼年特发性关节炎, 川崎病, 关节炎, 巨噬细胞活化综合征, 儿童

Abstract:

Objective Children with Kawasaki disease (KD) may have joint symptoms, and some children with systemic juvenile idiopathic arthritis (sJIA) present with Kawasaki disease-like symptoms at the onset, with similar manifestations in the early stage to KD. The early differentiation between these two groups of children is very difficult. This study aims to analyze the differences in early clinical features between the two types of children, and to explore the early warning signs of children who present with KD-like symptoms accompanied by joint manifestations but are actually diagnosed with sJIA. Methods A retrospective analysis was conducted on the clinical data of children who visited the Capital Institute of Pediatrics-Peking University Teaching Hospital from January 2021 to August 2025 and whose early symptoms met the diagnostic criteria for Kawasaki Disease (KD) with joint symptoms. All children completed at least 6 months of clinical follow-up. According to follow-up outcomes, patients were divided into the sJIA group (revised diagnosis of sJIA during the follow-up) and the KD group (maintaining KD diagnosis during the follow-up). Differences in fever characteristics, joint involvement patterns, inflammatory indicators, coronary artery lesions, and treatment response were compared between the two groups. Results Among 71 children with KD and joint symptoms, there were 46 boys and 25 girls, with a median onset age of 4.3 (2.5-5.1) years, a median fever duration of 9.0 (7.5-12.5) days, and a median interval from onset to joint symptoms appearance of 9.0 (6.5-12.0) days. Fifty-five children (77.5%) had oligoarthritis, 56 (78.9%) had large joint arthritis, only 2 had small joint arthritis, and 13 had mixed arthritis. The incidence of coronary artery aneurysm was 12.7% (9/71). One patient developed macrophage activation syndrome (MAS) during follow-up. All children received intravenous immunoglobulin (IVIg) treatment, and 32 children showed no response to initial IVIg treatment. After 6 months of follow-up, 8 children were finally diagnosed with sJIA (sJIA group) and 63 maintained the diagnosis of KD (KD group). The median duration of fever in the sJIA group was 24.5 days, which was significantly longer than 8.0 days in the KD group (P<0.01). There was no significant difference in CRP level at onset between the sJIA group and the KD group (P>0.05); the median CRP level in the KD group was significantly lower than that in the sJIA group after the first IVIg treatment (12.9 mg/L vs. 61.0 mg/L), and both ΔCRP and CRP reduction rate in the sJIA group were significantly lower than those in the KD group (all P<0.05). At 1-month follow-up, CRP level in the sJIA group was significantly higher than that in the KD group (P<0.001), while there was no significant difference in CRP level between the two groups at 6-month follow-up (P>0.05).At initial diagnosis, the median number of affected joints in the sJIA group was significantly higher than that in the KD group; The proportions of polyarthritis and mixed arthritis, the rates of wrist and interphalangeal joint involvement, and the incidence of synovial membrane thickening in the sJIA group were higher than those in the KD group, with statistically significant differences (all P<0.05). At 1-month and 6-month follow-up, the number of affected joints in the sJIA group was significantly higher than that in the KD group (both P<0.001). When duration of fever and number of affected joints were used as indicators to distinguish the sJIA group from the KD group, the AUC was 0.89 and 0.81, respectively. All sJIA children (100%, 8/8) showed no response to initial IVIg treatment, while the IVIg non-response rate in the KD group was 38.1% (24/63), with a statistically significant difference (P<0.05). There was no significant difference in the incidence of coronary artery lesions between the two groups (P>0.05). Conclusions Prolonged fever duration, polyarthritis or mixed arthritis, involvement of wrist and interphalangeal small joints, synovial membrane thickening on imaging, persistent joint symptoms, and no response to IVIg treatment are early clinical warning indicators for the final diagnosis of sJIA in KD children with joint symptoms.

Key words: systemic juvenile idiopathic arthritis, Kawasaki disease, arthritis, macrophage activation syndrome, child

中图分类号:

刘子尧, 王聪颖, 王宏茂, 张明明, 许瑛杰, 赖建铭, 牛文全, 李晓惠. 伴关节症状川崎病与川崎病样症状起病全身型幼年特发性关节炎的早期鉴别：一项单中心回顾性研究[J]. 临床儿科杂志, 2026, 44(6): 489-498.

LIU Ziyao, WANG Congying, WANG Hongmao, ZHANG Mingming, XU Yingjie, LAI Jianming, NIU Wenquan, LI Xiaohui. Early differentiation of Kawasaki disease with joint symptoms and systemic juvenile idiopathic arthritis with Kawasaki disease-like symptoms: a single-center retrospective study[J]. Journal of Clinical Pediatrics, 2026, 44(6): 489-498.

图/表 6

图1

表1

KD和sJIA组患儿临床特征比较"

项目	KD组(n=63)	sJIA组(n=8)	统计量	P
初诊时
起病年龄[M(P₂₅~P₇₅)]/岁	4.3(2.4~5.2)	4.0(3.3~4.5)	Z=0.32	0.750
男性[n(%)]	41(65.1)	5(62.5)	χ²=0.00	1.000
发热持续时间[M(P₂₅~P₇₅)]/d	8.0(7.0~11.0)	24.5(20.5~30.0)	Z=3.58	<0.001
起病至关节症状出现/发现时间[M(P₂₅~P₇₅)]/d	9.0(6.5~12.0)	12.5(6.5~18.5)	Z=1.11	0.266
Kobayashi评分[M(P₂₅~P₇₅)]	2.0(0.0~5.0)	5.0(3.5~6.0)	Z=1.57	0.116
CRP[M(P₂₅~P₇₅)]/mg·L^-1	87.0 (46.2~127.4)	70.7(57.0~107.4)	Z=0.45	0.656
IVIg治疗后CRP[M(P₂₅~P₇₅)]/mg·L^-1	12.9(4.5~30.1)	61.0(45.2~72.7)	Z=3.48	<0.001
ΔCRP[M(P₂₅~P₇₅)]/mg·L^-1	66.4(28.3~99.9)	15.2(9.0~34.2)	Z=2.89	0.011
CRP下降率[M(P₂₅~P₇₅)]/%	0.8(0.7~0.9)	0.2(0.1~0.5)	Z=3.88	<0.001
ESR[M(P₂₅~P₇₅)]/mm·h^-1	72.5(48.0~83.3)	74.5(69.3~75.5)	Z=0.27	0.789
铁蛋白[M(P₂₅~P₇₅)]/ng·mL^-1	300.7(214.3~376.1)	394.2(253.8~1152.2)	Z=1.16	0.246
纤维蛋白原[M(P₂₅~P₇₅)]/g·L^-1	5.4(4.5~6.8)	4.6(3.8~6.3)	Z=1.09	0.275
白细胞计数[M(P₂₅~P₇₅)]/×10⁹·L^-1	12.8(10.8~17.0)	9.9(7.7~13.2)	Z=1.61	0.108
血红蛋白( x±s)/g·L^-1	110.8±9.1	106.4±18.0	t=0.69	0.511
血小板计数[M(P₂₅~P₇₅)]/×10⁹·L^-1	367.0(291.0~463.5)	328.0(297.8~505.0)	Z=0.06	0.949
AST[M(P₂₅~P₇₅)]/U·L^-1	23.5(18.6~31.3)	32.5(28.1~59.2)	Z=2.25	0.025
ALT[M(P₂₅~P₇₅)]/U·L^-1	21.4(13.8~66.2)	22.3(17.2~112.6)	Z=0.49	0.623
LDH[M(P₂₅~P₇₅)]/U·L^-1	224.0(194.5~258.5)	230.5(214.5~282.5)	Z=0.76	0.445
IL-6[M(P₂₅~P₇₅)]/pg·mL^-1	33.7(14.1~173.0)	69.1(38.2~156.4)	Z=0.65	0.518
IL-1β[M(P₂₅~P₇₅)]/pg·mL^-1	9.0(5.0~16.5)	8.8(3.7~15.4)	Z=0.56	0.575
TNF-α[M(P₂₅~P₇₅)]/pg·mL^-1	23.1(14.6~32.8)	18.4(4.9~33.1)	Z=1.03	0.304
受累关节数[M(P₂₅~P₇₅)]/个	2.0(1.0~4.0)	5.5(3.75~20.0)	Z=2.91	0.004
冠状动脉瘤[n(%)]	8(12.7)	1(12.5)	χ²=0.00	1.000
MAS[n(%)]	0(0.0)	0(0.0)	－	－
随访1个月
CRP[M(P₂₅~P₇₅)]/mg·L^-1	4.9(1.3~8.9)	33.2(26.7~43.4)	Z=4.21	<0.001
受累关节数[M(P₂₅~P₇₅)]/个	0.0(0.0~1.0)	4.0(3.0~13.0)	Z=4.50	<0.001
冠状动脉瘤[n(%)]	6(9.5)	0(0.0)	－	1.000¹⁾
MAS[n(%)]	0(0.0)	1(12.5)	－	0.113¹⁾
随访6个月
CRP[M(P₂₅~P₇₅)]/mg·L^-1	1.7(0.6~3.7)	0.9(0.7~2.7)	Z=0.77	0.440
受累关节数[M(P₂₅~P₇₅)]/个	0.0(0.0~0.0)	3.0(1.0~8.0)	Z=5.14	<0.001
冠状动脉瘤[n(%)]	1(1.7)	0(0.0)	－	1.000¹⁾
MAS[n(%)]	0(0.0)	0(0.0)	－	－

表1

图2

表2

表3

表4

参考文献 32

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项目	KD组(n=63)	sJIA组(n=8)	χ²值	P
按关节症状出现/发现时间分类			0.11	0.739
早发型关节炎	32(50.8)	3(37.5)
晚发型关节炎	31(49.2)	5(62.5)
按关节数量分类			5.87	0.015
少关节炎	52(82.5)	3(37.5)
多关节炎	11(17.5)	5(62.5)
按关节种类分类			－	<0.001¹⁾
大关节炎	55(87.3)	1(12.5)
小关节炎	2(3.2)	0(0.0)
混合型关节炎	6(9.5)	7(87.5)
具体受累关节
髋	37(58.7)	7(87.5)	1.42	0.233
膝	34(53.97)	7(87.5)	2.04	0.153
踝	20(31.8)	3(37.5)	0.00	1.000
腕	8(12.7)	4(50.0)	4.63	0.031
肘	3(4.76)	0(0.0)	－	1.000¹⁾
肩	3(4.76)	1(12.5)	－	0.387¹⁾
指	5(7.9)	4(50.0)	7.86	0.005
趾	2(3.2)	1(12.5)	－	0.305¹⁾
影像学表现
关节积液	52(82.5)	6(75.0)	0.00	0.973
滑膜增厚	16(25.4)	6(75.0)	6.01	0.014
软组织水肿	12(19.1)	3(37.5)	0.55	0.456
骨髓水肿	0(0.0)	1(12.5)	－	0.113¹⁾
骨质破坏	0(0.0)	0(0.0)	－	－

项目	KD组(n=63)	sJIA组(n=8)	χ²值	P
IVIg	63(100.0)	8(100.0)	－	－
IVIg无反应	24(38.1)	8(100.0)	8.63	0.003
DMARDs	0(0.0)	8(100.0)	－	<0.001^1）
糖皮质激素	25(39.7)	8(100.0)	8.10	0.004
生物制剂	5(7.9)	6(75.0)	19.53	<0.001
英夫利昔单抗	5(7.9)	2(25.0)	－	0.175^1）
托珠单抗	0(0.0)	3(37.5)	－	<0.001^1）
托法替布	0(0.0)	1(12.5)	－	0.113^1）
治疗组合
IVIg单用	35(55.6)	0(0.0)	6.68	0.010
IVIg+糖皮质激素	23(36.5)	0(0.0)	2.81	0.093
IVIg+生物制剂	3(4.8)	0(0.0)	－	1.000^1）
IVIg+糖皮质激素+生物制剂	2(3.2)	0(0.0)	－	1.000^1）
IVIg+糖皮质激素+DMARDs	0(0.0)	2(25.0)	－	0.011^1）
IVIg+生物制剂+DMARDs	0(0.0)	0(0.0)	－	－
IVIg+糖皮质激素+生物制剂+DMARDs	0(0.0)	6(75.0)	－	<0.001^1）

项目	KD组(n=63)	sJIA组(n=8)	统计量	P
无冠状动脉受累[n(%)]	54(85.7)	6(75.0)	χ²=0.07	0.787
冠状动脉扩张[n(%)]	1(1.6)	1(12.5)	－	0.214^1）
冠状动脉瘤分型[n(%)]
小型	6(9.5)	1(12.5)	－	0.584^1）
中型	2(3.2)	0(0.0)	－	1.000^1）
巨大型	0(0.0)	0(0.0)	－	－
冠状动脉各分支Z值[M(P₂₅~P₇₅)]
LM	0.89(0.37~1.65)	1.12(0.86~1.33)	Z=0.53	0.599
LAD	0.47(－0.03~1.06)	0.51(0.30~0.89)	Z=0.25	0.799
LCX	－0.12(－0.52~0.55)	－0.33(－0.64~－0.01)	Z=0.96	0.335
RCA	0.52(－0.16~0.91)	0.11(－1.58~0.50)	Z=1.60	0.109

伴关节症状川崎病与川崎病样症状起病全身型幼年特发性关节炎的早期鉴别：一项单中心回顾性研究

Early differentiation of Kawasaki disease with joint symptoms and systemic juvenile idiopathic arthritis with Kawasaki disease-like symptoms: a single-center retrospective study

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