多发性大动脉炎合并肺动脉高压临床特点分析  

Analysis of clinical features of Takayasu＇s arteritis complicated with pulmonary arterial hypertension

作　　者：万瑾[1] 王天[1] 潘丽丽[1] 慈维苹[1] 廖华[1] 

机构地区：[1]首都医科大学附属北京安贞医院风湿免疫科,100029

出　　处：《中国医药》2015年第10期1435-1438,共4页China Medicine

基　　金：国家自然科学基金（81400361）;首都医科大学基础临床合作基金（14JL64）

摘　　要：目的 探讨多发性大动脉炎（TA）合并肺动脉高压（PAH）的临床特点,以提高对该病的认识.方法 回顾性选择2004年1月至2014年1月首都医科大学附属北京安贞医院收治的TA患者85例,分析患者的临床表现、实验室检查及影像学资料,比较合并与未合并PAH的TA患者临床资料.结果 85例TA患者中7例（8.2％）合并PAH,其中男1例,女6例;7例有肺动脉受累而无PAH,二者发病年龄、平均确诊时间比较[（25±3）岁比（33±7）岁,（55±41）个月比（19±8）个月],差异有统计学意义（P＜0.05）.7例TA合并PAH患者中4例以胸背痛、活动后气短为首发症状;受累血管主要为锁骨下动脉（5例）、肺动脉（4例）和主动脉（4例）.临床分型Ⅰ型1例,Ⅲ型2例,Ⅱ＋Ⅳ型1例,Ⅲ＋Ⅳ型2例,Ⅰ＋Ⅳ型1例;肺动脉狭窄4例,心脏瓣膜病变6例,平均肺动脉收缩压（62±22） mmHg（1 mmHg=0.133 kPa）;药物治疗中单独使用激素治疗2例,激素联合环磷酰胺、阿司匹林及来氟米特治疗1例,雷公藤联合阿司匹林治疗1例,单独抗血小板治疗3例;C反应蛋白[（16±4） mg/L]、红细胞沉降率[（45±7） mm/1 h]均高于正常值;TA合并PAH患者较不合并PAH患者易出现活动后气短、乏力,不易出现头晕,易有肺动脉、升主动脉及主动脉弓受累[57.1％ （4/7）比20.5％（16/78）,71.4％ （5/7）比34.6％ （27/78）,0.0％ （0/7）比64.1％ （50/78）,57.1％ （4/7）比9.0％（7/78）,28.6％（2/7）比9.0％（7/78）,28.6％（2/7）比3.8％ （3/78）],差异有统计学意义（P＜0.05）.结论 PAH是TA累及肺动脉时出现的严重并发症.临床医师应关注出现活动后气短、乏力以及肺动脉、升主动脉、主动脉弓受累的TA患者的肺动脉压力情况,积极治疗原发病有助于降低肺动脉收缩压.Objective To explore the clinical features of Takayasu＆#39;s arteritis （TA） complicated with pulmonary arterial hypertension （PAH）.Methods Clinical manifestations,lab data and imaging findings of 85 TA inpatients from January 2004 to January 2014 were retrospectively analyzed.The clinical data were compared between patients with PAH and without PAH.Results Seven patients （8.2%） had PAH,including 6 females and 1 male.Seven patients had pulmonary artery involved but without PAH and their onset age and time of diagnosis were significantly different from those TA patients with PAH [（33 ±7） years vs （25 ±3） years,（19 ±8） months vs （55 ±41） months] （P 〈0.05）.In patients with PAH,4 cases had chest/back pain and anhelation after exercise as initial symptoms;the involved vessels were mainly subclavian artery （5 cases）,pulmonary artery （4 cases） and aorta （4 cases）;according to angiographic classification,1 case was type Ⅱ,2 cases were type Ⅲ,1 case was type Ⅱ ＋ Ⅳ,2 cases were type Ⅲ ＋ Ⅳ,1 case was type Ⅰ ＋ Ⅳ;6 cases had pulmonary stenosis;6 cases had valve lesions;the average pulmonary arterial systolic pressure was （62 ± 22） mmHg.Regarding drug treatment,single corticosteroid treatment was given in 2 cases;corticosteroid combined with cyclophosphamide,asprin and leflunomide were given in 1 case;tripterygium combined with asprin were given in 1 case;single antiplatelet therapy was given in 3 cases;the level of C reactive protein and erythrocyte sedimentation rate were both abnormally increased [（16 ±4） mg/L,（45 ±7） mm/1 h].Compared with patients without PAH,patients with PAH were more prone to anhelation after exercise,weakness,and there were more involvement of pulmonary artery,ascending aorta and aortic arch but less dizziness [57.1% （4/7） vs 20.5% （16/78）,71.4% （5/7） vs 34.6% （27/78）,57.1% （4/7）vs9.0% （7/78）,28.6% （2/7）vs9.0% （7/78）,28.6% （2/7）vs3.8%（3/78）,0.0% （0/7） vs 64.1% （50/78）]

关 键 词：大动脉炎 肺动脉高压 临床特点 

分 类 号：R543.5[医药卫生—心血管疾病]