摘要
目的分析结缔组织病（connectivetissuedisease，CTD）相关间质性肺疾病（interstitiallungdisease，ILD）继发肺动脉高压（pulmonaryhypertension，PH）的临床表现、肺功能、肺部CT／HRcT、血气及实验室检查等临床资料，探讨CTD-ILD继发PH的特点。方法回顾性分析557例CTD-ILD患者的临床资料，比较CTD-ILD-PH组与CTD-ILD组的临床特点。结果①CTD继发ILD的发病率为27．65％，CTD-ILD发生PH的发病率为13．11％；②CTD-ILD继发PH原发病的发病率由高到低依次为：重叠综合征、混合性结缔组织病、系统性硬化症、系统性红斑狼疮、原发性干燥综合征、多发性肌炎／皮肌炎及类风湿关节炎；③CTD-ILD-PH组患者咳痰、气短、呼吸困难、雷诺现象、皮肤变硬的发生率及静息心率均高于CTI）_ILD组（P值均〈O．05）；④CTD-ILD-PH组的肺部CT／HRCT中磨玻璃密度影、网格影、小叶间隔增厚、肺动脉干增粗、心影增大及胸腔积液的发生率高于CTD-ILD组（P值均〈0．05）；⑤CTD-ILD-PH组肺功能指标FVC％pred、DLC0％pred及血气指标Pa0：均低于CTD-ILD组（P值均d0．05）；⑥CTD-ILD-PH组心脏彩超提示右室内径、右室流出道内径、肺动脉内径及三尖瓣反流速度高于CTD-ILD组（P值均d0．05）；⑦ANA抗体及SM抗体阳性者更易继发PH。结论①CTD继发ILD的发病率为27．65％，CTD-ILD发生PH的发病率为13．11％。②重叠综合征、混合性结缔组织病合并ILD时较其他CTD患者更易继发PH；③当CTD-ILD患者出现咳痰、气短、呼吸困难、雷诺现象及心率增快症状时，应完善相关检查，警惕PH的发生；④肺CT／HRCT发现网格影、小叶间隔增厚、肺动脉干增粗及心影增大或心脏超声提示右室大、三尖瓣反流速度增高，应监测肺动脉压；⑤CTD-ILD患者肺功能FVC％pred、Dt。CO％pred及血气PaOz下降明显时，注意发生PH；⑥ANA及SM抗体可作为预测CT胁ILD-PH简单、易行的指标。
Objective To study the clinical features of connective tissue disease （CTD） with interstitial lung disease （ILD） complicating pulmonary hypertension （PH）. Methods The clinical data of 557 cases of CTD-ILD were retrospectively analyzed, the clinical characteristics of CTD-ILD-PH group and CTD-ILD group were compared. Results （1） The incidence rate of ILD secondary to CTD was 27.65%,the prevalence rate of CTD-ILD-induced PH was 13.11%. （2）The primary diseases in PH secondary to CTD-ILD according to prevalence rate from high to low were： overlap syndrome, mixed connective tissue disease, systemic sclerosis, systemic lupus erythematosus, primary desiccation syndrome, polymyositis/dermatomyositis, and rheumatoid arthritis. （3） The incidence rates of expectoration, breathless,dyspnea,Raynaud＇s phenomenon, skin hardens, and resting heart rate of CTD-ILD-PH group were higher than those of CTD-ILD group （all P d0.05）. （4）The incidence rates of ground-glass opacity, grid shadow,interlobular septal thickening, thickening of pulmonary artery, heart enlargement and pleural effusion in pulmonary CT/HRCT of CTD-ILD-PH group were higher than those of CTD-ILD group （all P 〈0.05）. （5）FVCMpred,DLCO%pred,and PaO2 in CTD-ILD-PH group were lower than those in CTD-ILD group （all P 〈 0.05）. （6）The echoeardiography showed that the incidence rates of right ventrieular diameter, right ventricular outflow tract diameter, pulmonary artery diameter and three tricuspid regurgitation velocity in CTD-ILD-PH group were higher than those in CTD-ILD group （all P d0.05）. （7） The positive ANA and SM antibodies were prone to secondary PH. Conclusions （1）The incidence rate of ILD secondary to CTD is 27. 65%, the prevalence rate of CTD-ILD-induced PH is 13.11 %. （2）Overlap syndrome or mixed connective tissue disease combined with ILD is more likely to complicating PH than other CTD. （3）When the CTD-ILD patients have expectoration, breathless, dyspnea, Raynaud＇ s phenomenon,skin hardens and increased heart rate, the relevant inspection should be improved, the occurrence of PH should be alerted. （4） When the lung CT/HRCT shows grid shadow,interlobular septal thickening, pulmonary artery thickening or cardiac ultrasound shows right ventricle enlargement or increased three tricuspid regurgitation velocity,the pulmonary artery pressure should monitored. （5）When FVC%pred,DLCO% pred and PaO2 obviously decrease in patients with CTD-ILD, PH should be paied attention to. （6） ANA and SM antibodied can be used to predict CTD-ILD-PH as simple and feasible indexes.
出处
《国际呼吸杂志》
2015年第16期1213-1218,共6页
International Journal of Respiration
关键词
结缔组织病
间质性肺疾病
肺动脉高压
心脏彩超
肺CT/HRCT
肺功能
Connective tissue disease
Interstitial lung disease
Pulmonary hypertension
Ultrasoniccardiogram
Pulmonary CT/HRCT
Lung function
