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

Abstract

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

CLC Number:

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.

Figures/Tables 6

Figure 1

Table 1

Comparison of clinical characteristics between KD group and sJIA group"

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)	－	－

Table 1

Figure 2

Table 2

Table 3

Table 4

References 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

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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