伴肺动脉高压的大动脉炎临床及影像学特征研究

Clinical features and CT findings of Takayasu′s arteritis associated pulmonary hypertension

目的探讨大动脉炎累及肺动脉(PTA)伴肺动脉高压(PH)患者的临床及影像学特征。方法回顾性分析空军军医大学第一附属医院2008年1月至2018年1月间确诊为PTA的40例患者,其中伴PH患者14例(PTA+PH组),不伴PH的PTA患者26例(PTA组),分别采用χ2或Fisher精确检验、两独立样本t检验以及Mann-Whitney U秩和检验比较两组的一般资料、症状、体征、实验室检查结果、右心及肺动脉测量数据和CT血管成像(CTA)表现。结果与PTA组比较,PTA+PH组患者病程时间更长[48.0(18.5,73.5)个月比24.5(10.5,48.5)个月],活动期患者占比较低(4/14比17/26),多存在气短、气喘、胸闷症状和下肢水肿体征,PaO_(2)较低,ESR较慢,右心房及右心室宽度增加(均P<0.05)。患者肺动脉受累的主要CT征象包括管腔狭窄(39/40)、管腔闭塞(16/40)、管壁增厚(9/40)和管腔扩张(2/40)。PTA+PH组管腔重度狭窄(≥50%)比例高于PTA组,管壁增厚和管腔轻度狭窄(<50%)比例较低(均P<0.05)。结论伴有PH的PTA患者的临床表现、实验室检查结果及CTA均可见特征性表现,可能与疾病所处的病程阶段、严重程度、是否有活动性、预后等有相关性,临床应给予重视。

Objective Takayasu′s arteritis involving the pulmonary artery(PTA)is uncommon,and those with pulmonary hypertension(PH)are even rarer.This study investigated the clinical features and CT findings in PTA patients with PH.Methods A total of 40 PTA patients were retrospective selected in the First Hospital of Air Force Medical University from January 2008 to January 2018.There were 14 PTA patients with PH,including 3 male and 11 female cases,aged from 18 to 53(29.7±9.4)years,as the study group(PTA+PH group).There were 26 PTA patients without PH,including 4 males and 22 females,aged 15-52(28.9±8.5)years,as the control group(PTA group).The Chi-square or Fisher′s test,T test of two independent samples and Mann-Whitney U rank sum test were used to compare the general information,symptoms,signs,laboratory examination data,right ventricular and pulmonary artery measurement data,and pulmonary artery CT findings between the two groups.Results Compared with the PTA group,the patients in the PTA+PH group had longer disease duration,fewer active cases,more shortness of breath,chest distress and lower limb edema,lower blood oxygen partial pressure(PaO_(2))and lower ESR(all P<0.05).The width of right atrium and right ventricle in PTA+PH group was greater than that in PTA group(all P<0.05).The main CT findings of the involved pulmonary artery included lumen stenosis(39 cases,97.5%),lumen occlusion(16 cases,40%),wall thickening(9 cases,22.5%),and lumen dilation(2 cases,5.0%).Patients in the PTA+PH group had less wall thickening and mild lumen stenosis(<50%),more severe lumen stenosis(≥50%)and occlusion than those in the PTA group(all P<0.05).Conclusions PTA patients with PH showed certain characteristics in clinical,laboratory and CT findings,which may be correlated to the stage of the disease duration,the severity,and the prognosis.