摘要
目的 探讨全身型幼年特发性关节炎(sJIA)合并巨噬细胞活化综合征(MAS)患儿的临床特征及远期预后。方法 回顾性分析2014年8月-2020年8月收治的sJIA合并MAS患儿的临床资料,将同期诊治的sJIA无合并MAS患儿作为sJIA无合并MAS组,比较两组间临床表现和远期预后。结果 sJIA合并MAS组25例中男11例,女14例,中位起病年龄5.8(2.6~10.0)岁。s JIA无合并MAS组38例中,男18例、女20例,中位起病年龄7.0(5.1~9.0)岁。21例患儿在sJIA病程早期合并MAS。与sJIA无合并MAS组相比,sJIA合并MAS组肝脾肿大、呼吸系统受累、中枢神经系统受累及出血表现的发生率较高,差异有统计学意义(P<0.05)。sJIA合并MAS患儿实验室指标异常以高铁蛋白血症最为常见(100%),其次为天冬氨酸氨基转氨酶升高(24例)、纤维蛋白原降低(23例)。所有患儿均使用了糖皮质激素治疗,22例静脉加用丙种球蛋白,22例加用环孢素A;11例加用托珠单抗,其中4例在MAS活动期2~3周内应用,7例在MAS缓解后sJIA病情不稳定时加用。中位随访时间为40.0(26.5~60.5)月。随访至2022年8月31日的结果显示,sJIA合并MAS组单相病程11例,反复病程5例,持续病程9例,与sJIA无合并MAS组远期预后差异无统计学意义(P>0.05)。4例接受糖皮质激素治疗持续时间长的sJIA合并MAS患儿出现生长迟缓,3例sJIA合并MAS患儿出现激素性白内障。结论 早期识别sJIA合并MAS,并及时给予有效治疗、规范管理,其远期预后与无合并MAS患儿相当。
Objective To investigate the clinical features and long-term prognosis of systemic juvenile idiopathic arthritis(sJIA)complicated with macrophage activation syndrome(MAS).Methods The clinical data of sJIA children complicated with MAS treated from August 2014 to August 2020 were retrospectively analyzed.The sJIA children without MAS diagnosed and treated in the same period were classified as sJIA without MAS group.The clinical manifestations and long-term prognosis were compared between the two groups.Results The median onset age of the 25 patients(11 boys and 14 girls)in sJIA with MAS group was 5.8(2.6-10.0)years.The median onset age of 38 patients(18 boys and 20 girls)in sJIA without MAS group was 7.0(5.1-9.0)years.Twenty-one patients had MAS in the early stage of sJIA.Compared with the sJIA without MAS group,the incidence of hepatosplenomegaly,respiratory system involvement,central nervous system involvement and bleeding in the sJIA with MAS group was higher,and the difference was statistically significant(P<0.05).Methemoglobinemia was the most common abnormal laboratory index in sJIA patients with MAS(100%),followed by elevated aspartate aminotransferase(24 cases)and decreased fibrinogen(23 cases).All patients were treated with glucocorticoids,22 with intravenous immunoglobulin and 22 with cyclosporin A.Tocilizumab was added to 11 patients,4 of them were treated with tocilizumab within 2 to 3 weeks of MAS activity,and 7 were treated with tocilizumab when sJIA was still in an unstable state after MAS remission.The median follow-up time was 40.0(26.5-60.5)months.The results of follow-up to August 31,2022 showed that there were 11 cases of monophasic course,5 cases of recurrent course,and 9 cases of persistent course in the sJIA with MAS group,and there was no statistically significant difference in long-term prognosis compared with the sJIA without MAS group(P>0.05).Growth retardation occurred in 4 sJIA children with MAS who received glucocorticoid therapy for a long duration.Hormonal cataract occurred in 3 sJIA children with MAS.Conclusions For sJIA children complicated with MAS,if early identification,effective treatment and standardized management can be given in time,the long-term prognosis is similar to that of children without MAS.
作者
刘平
江春亚
魏磊
王向丽
靳云凤
秦玉洁
杨亚妮
刘翠华
LIU Ping;JIANG Chunya;WEI Lei;WANG Xiangli;JIN Yunfeng;QING Yujie;YANG Yani;LIU Cuihua(Department of Nephrology and Rheumatology,Children's Hospital Affiliated to Zhengzhou University,Henan Provincial Children's Hospital,Zhengzhou Children's Hospital,Zhengzhou 450000,Henan,China)
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2024年第3期218-223,共6页
Journal of Clinical Pediatrics
基金
河南省医学科技攻关项目(No.LHGJ20210623)。
关键词
全身型幼年特发性关节炎
巨噬细胞活化综合征
临床特征
远期预后
systemic juvenile idiopathic arthritis
macrophage activation syndrome
clinical feature
long-term prognosis
