全身型幼年型特发性关节炎合并冠状动脉扩张5例临床分析

Clinical analysis of 5 cases of systemic juvenile idiopathic arthritis with coronary artery dilatation

目的探讨全身型幼年型特发性关节炎合并冠状动脉扩张的临床特点。方法回顾性分析2019年5月至2021年6月在首都儿科研究所附属儿童医院风湿免疫科住院的5例全身型幼年型特发性关节炎合并冠状动脉扩张患儿的病例资料,分析临床表现、血常规、炎症指标、超声心动图、血管超声及CT血管造影、治疗转归等情况。结果5例患儿中男2例、女3例,起病年龄为7月龄至4岁7月龄,诊断时间为1.5~3.0个月,其中4例曾被诊断为不完全川崎病。3例为单侧冠状动脉扩张,2例为双侧冠状动脉扩张。4例合并多器官损伤、3例合并巨噬细胞活化综合征、3例合并肺损伤、2例合并心包积液、1例合并肺动脉高压。3例患儿应用甲泼尼龙冲击治疗并予以甲氨蝶呤联合环孢素,最终应用生物制剂后病情得到控制并已减停泼尼松,另2例应用足量泼尼松口服治疗并逐渐减量,同时加用甲氨蝶呤,1例在泼尼松减量过程中复发。5例患儿均未发现其他血管受累。冠状动脉扩张在治疗1~3个月后完全恢复。结论全身型幼年型特发性关节炎合并冠状动脉扩张者起病年龄小,诊断时间长,易出现多器官损伤,对激素和常规免疫抑制剂不敏感,应尽早加用生物制剂治疗。如治疗及时,冠状动脉扩张预后良好。

Objective To investigate the clinical characteristics of systemic juvenile idiopathic arthritis combined with coronary artery dilatation.Methods A retrospective analysis was performed on the clinical data,including clinical manifestations,blood routine,inflammatory factors,echocardiography,vascular ultrasound and CT angiography,treatment and outcomes,etc,of 5 cases with systemic juvenile idiopathic arthritis combined with coronary artery dilation admitted to Department of Rheumatology in the affiliated Children′s Hospital of Capital Institute of Pediatrics from May 2019 to June 2021.Results There were 2 males and 3 females among 5 cases.The onset age ranged from 7 months to 4 years 7 months.The diagnostic time ranged from 1.5 months to 3.0 months.Four cases were diagnosed as atypical Kawasaki disease.Three cases showed unilateral coronary artery dilation.Two cases showed bilateral coronary artery dilation.Four cases developed multiple organ injuries.Three cases developed macrophage activation syndrome.Three cases developed lung injury.Two cases developed pericardial effusion.One case developed pulmonary hypertension.As for treatment,3 cases treated with methylprednisolone pulse therapy and methotrexate combined with cyclosporine,improved after the final application of biological agents,and have stopped prednisone.The other 2 cases were treated with adequate oral prednisone and gradually reduced,and methotrexate was added at the same time,1 case relapsed in the process of reduction.No other vascular involvement was found in 5 cases.Coronary artery dilation recovered completely after 1 to 3 months of treatment.Conclusions Systemic juvenile idiopathic arthritis combined with coronary artery dilatation has the clinical characteristics of small onset age,long diagnostic time,prone to multiple organ injuries.Corticosteroids and conventional immunosuppressive agents are not sensitive,and biological agents should be used as soon as possible.The prognosis of coronary artery dilation is good after timely treatment.