以川崎病样症状起病的全身型幼年特发性关节炎临床分析  

Clinical analysis of systemic juvenile idiopathic arthritis with Kawasaki disease-like symptoms

作　　者：王迪 罗冲[1] 唐雪梅[1] 周娟[1,2] Wang Di;Luo Chong;Tang Xuemei;Zhou Juan(Department of Rheumatology and Immunology,Children′s Hospital of Chongqing Medical University,National Clinical Research Center for Child Health and Disorders,Ministry of Education Key Laboratory of Child Developmental Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院风湿免疫科、国家儿童健康与疾病临床研究中心、儿童发育疾病研究教育部重点实验室、儿科学重庆市重点实验室,重庆400014 [2]广州医科大学附属妇女儿童医疗中心过敏免疫缺陷风湿病科,广州510630

出　　处：《中华儿科杂志》2024年第12期1158-1163,共6页Chinese Journal of Pediatrics

摘　　要：目的分析以川崎病样症状起病的全身型幼年特发性关节炎(sJIA)患儿的临床特点。方法病例对照研究。选择2018年1月至2024年8月重庆医科大学附属儿童医院风湿免疫科24例以川崎病样症状起病的sJIA患儿为川崎病合并sJIA组,选取同科室同期96例川崎病患儿为川崎病组,83例sJIA患儿为sJIA组,收集3组间一般资料、临床表现、实验室检查及并发症资料。组间比较采用Mann-Whitney U或Kruskal-Wallis H检验、χ^(2)检验或Fisher确切概率法。结果川崎病合并sJIA组、川崎病组、sJIA组之间起病年龄、热程比较差异均有统计学意义[3.4(2.5,7.3)比3.4(1.9,4.8)比8.8(5.1,11.7)岁,24.5(18.0,37.3)比23.0(18.0,31.0)比7.0(6.0,8.0)d,Z=67.09、138.24,均P<0.05];川崎病合并sJIA组24例患儿中,20例(83%)存在关节症状,9例(38%)结膜充血;川崎病合并sJIA组、川崎病组、sJIA组发生冠状动脉损伤率比较,差异有统计学意义[58%(14/24)比44%(42/96)比6%(5/83),χ2=40.50,P<0.05];川崎病合并sJIA组sJIA组及川崎病组发生巨噬细胞活化综合征风险的比例,差异有统计学意义[17%(4/24)比10%(8/83)比0,P<0.05]。川崎病合并sJIA组24例患儿中,接受2个疗程静脉注射免疫球蛋白(IVIG)治疗仍无应答11例(46%),应用糖皮质激素21例(88%)。川崎病合并sJIA组大剂量激素使用率高于sJIA组[29%(7/24)比5%(4/83),χ2=12.95,P<0.05]。川崎病合并sJIA组中,使用生物制剂17例(71%),1例使用阿达木单抗,16例接受托珠单抗治疗(托珠单抗过敏4例);11例(46%)托珠单抗治疗者规律随访1个月,10例有效。结论以川崎病样临床症状起病的sJIA患儿,有川崎病与sJIA的临床特征,对起病年龄小、热程偏长,伴有关节症状,IVIG耐药的川崎病患儿需及时警惕sJIA可能,及时予以激素及生物制剂治疗。ObjectiveTo analyze the clinical characteristic of systemic juvenile idiopathic arthritis(sJIA)patients with Kawasaki disease like onset symptom.MethodsA case-control study was performed.A total of 24 patients with sJIA with Kawasaki disease-like symptoms at the Department of Rheumatology and Immunology,Children′s Hospital of Chongqing Medical University from January 2018 to August 2024 were selected as the Kawasaki disease combined with sJIA group.A total of 96 patients with Kawasaki disease as the Kawasaki disease group and 83 patients with sJIA were selected as the sJIA group.The general information,clinical manifestations,laboratory examinations and complications of the patients were compared among the 3 groups.Differences between groups were assessed by Mann-Whitney U test or Kruskal-Wallis H test and Chi-square test or Fisher's exact test.ResultsThere were significant differences in age and fever course between Kawasaki disease combined with sJIA groups,Kawasaki disease groups,and sJIA groups(3.4(2.5,7.3)vs.3.4(1.9,4.8)vs.8.8(5.1,11.7)years,24.5(18.0,37.3)vs.23.0(18.0,31.0)vs.7.0(6.0,8.0)d,Z=67.09,138.24,both P<0.05).Among the 24 cases of Kawasaki disease combined with sJIA,20 cases(83%)had joint symptoms and 9 cases(38%)had conjunctival congestion.There were significant differences in the incidence of coronary artery injury between Kawasaki disease combined with sJIA group,Kawasaki disease group and sJIA group(58%(14/24)vs.44%(42/96)vs.6%(5/83),χ2=40.50,P<0.05).There were significant differences in the risk of macrophage activation syndrome between Kawasaki disease combined with sJIA group,sJIA group and Kawasaki disease group(17%(4/24)vs.10%(8/83)vs.0,P<0.05).In the Kawasaki disease combined with sJIA group,11 cases(46%)did not respond after 2 courses of intravenous immunoglobulin(IVIG)treatment,and 21 cases(88%)used glucocorticoids.The use rate of high-dose hormones in the Kawasaki disease combined with sJIA group was higher than that in the sJIA group(29%(7/24)vs.5%(4/83),χ2=12.95,P<0.05).In the gro

关 键 词：黏膜皮肤淋巴结综合征 关节炎 并发症 治疗 

分 类 号：R725.4[医药卫生—儿科]