儿童风湿病合并肺动脉高压随访及预后分析  

Follow-up and prognosis analysis of rheumatic disease complicated with pulmonary arterial hypertension in children

作　　者：朱佳[1] 包敏[2] 刘小惠[3] 徐莉燕[4] 张曼[4] 贺宝平 许瑛杰[1] 赖建铭[1] Zhu Jia;Bao Min;Liu Xiaohui;Xu Liyan;Zhang Man;He Baoping;Xu Yingjie;Lai Jianming(Department of Rheumatology and Immunology,Children′s Hospital Affiliated to the Capital Institute of Pediatrics,Beijing 100020,China;Department of Cardiovascular,Children′s Hospital Affiliated to the Capital Institute of Pediatrics,Beijing 100020,China;Department of Rheumatology and Immunology,Jiangxi Children′s Hospital,Nanchang 330000,China;Department of Pediatrics I,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450000,China;Department of Pediatrics,the Affiliated Hospital of Inner Mongolia Medical University,Hohhot 010000,China)

机构地区：[1]首都儿科研究所附属儿童医院风湿免疫科,北京100020 [2]首都儿科研究所附属儿童医院心血管内科,北京100020 [3]江西省儿童医院风湿免疫科,南昌330000 [4]郑州大学第一附属医院儿科一,郑州450000 [5]内蒙古医科大学附属医院儿科,呼和浩特010000

出　　处：《中华实用儿科临床杂志》2022年第24期1873-1878,共6页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的探讨儿童风湿病合并肺动脉高压(PAH)的临床特点、随访特征及预后情况,为临床诊治提供参考意见。方法回顾性分析2013年1月至2022年6月在首都儿科研究所附属儿童医院风湿免疫科、江西省儿童医院风湿免疫科、郑州大学第一附属医院儿科一、内蒙古医科大学附属医院儿科住院的风湿病合并PAH 24例患儿资料。于2022年6月30日对合并PAH的病例进行电话随访,收集临床症状、治疗、随访、预后情况等。根据不同治疗方式分为不同的临床亚组,分析PAH变化情况,组间比较采用t检验,P<0.05为差异有统计学意义。结果共纳入24例患儿,其中男7例,女17例;出现PAH的年龄为(10.97±3.79)岁;出现PAH时病程中位数为6.00(32.20)个月;平均肺动脉压为(51.71±17.66)mmHg(1 mmHg=0.133 kPa)。其中,系统性红斑狼疮9例,多发性大动脉炎5例,幼年皮肌炎、未分化结缔组织病各3例,幼年特发性关节炎全身型2例,白塞病、川崎病各1例。24例患儿常见的症状有发热(14例)、乏力(10例)、呼吸困难(7例)。24例患儿合并心包积液10例,瓣膜反流9例,收缩和/或舒张心功能减低5例。24例患儿中,17例出现肺部改变。共11例患儿进行B型尿钠肽检测,均升高,中位数为3073(10645)ng/L。首次出现PAH后,在原发病治疗中有12例进行甲泼尼龙治疗,10例行环磷酰胺治疗,2例(均为系统性红斑狼疮)行血液净化治疗。PAH治疗中,11例行降肺动脉压治疗,其中7例使用PAH靶向药物治疗,使用靶向治疗的患儿平均肺动脉压降低值为(44.80±24.08)mmHg,未使用靶向治疗的患儿降低值为(16.15±17.25)mmHg,差异有统计学意义(t=2.661,P=0.016)。20例患儿完成复查和/或随访,随访病程为(36.29±26.67)个月:6例平均肺动脉压完全恢复,恢复时间中位数8.00(13.47)个月,但其中2例在肺动脉压完全恢复后死亡;11例平均肺动脉压较前好转,其中1例死亡,其余病情稳定;2例患儿平均肺动脉压�Objective To explore the clinical features,follow-up characteristics and prognosis of rheumatic disease complicated with pulmonary arterial hypertension(PAH)in children,and to provide support for its clinical diagnosis and treatment.Methods A retrospective analysis was conducted on the data of rheumatic 24 patients complicated with PAH hospitalized in the Department of Rheumatology and Immunology,Children′s Hospital Affiliated to the Capital Institute of Pediatrics,Department of Rheumatology and Immunology,Jiangxi Children′s Hospital,Department of PediatricsⅠ,the First Affiliated Hospital of Zhengzhou University and Department of Pediatrics,the Affiliated Hospital of Inner Mongolia Medical University from January 2013 to June 2022.The rheumatic patients complicated with PAH were followed up by telephone on June 30,2022,and their clinical symptoms,treatment,follow-up,and prognosis data were collected.According to different treatment methods,the patients were divided into different clinical subgroups.The change of PAH was analyzed.The t-test was used for comparison between groups.P<0.05 was statistically significant.Results A total of 24 cases were enrolled,with 7 males and 17 females.The average onset age of PAH was(10.97±3.79)years old.The median duration of PAH was 6.00(32.20)months.The average pulmonary artery pressure was(51.71±17.66)mmHg(1 mmHg=0.133 kPa).There were 9 cases of systemic lupus erythematosus,5 cases of Takayasu′s arteritis,3 cases of juvenile dermatomyositis,3 cases of undifferentiated connective tissue disease,2 cases of systemic juvenile idiopathic arthritis,1 case of Behcet′s disease,and 1 case of Kawasaki disease.Among 24 cases,the common symptoms were fever(14 cases),fatigue(10 cases)and dyspnea(7 cases).Of the 24 cases,10 cases were complicated with hydropericardium,9 cases with valve regurgitation,and 5 cases with decreased systolic and/or diastolic function.Lung changes were observed in 17 cases.Eleven cases were tested for B-type natriuretic peptide(BNP),and the BNP levels w

关 键 词：风湿病 儿童 肺动脉高压 随访 预后 

分 类 号：R725[医药卫生—儿科]