系统性硬化症的肺部表现

Pulmonary Involvement in Patients with Systemic Sclerosis

目的分析系统性硬化症(SSc)患者的肺部受累特点及与功能性指标的相关性。方法前瞻性序贯入组68例SSc患者。所有患者完成病史采集和查体,胸部高分辨CT(HRCT),肺功能[用力肺活量(FVC)、肺总量(TLC)、肺一氧化碳弥散量(DLCO)],6分钟步行试验,以及心脏彩色多普勒检查。其中36例完成圣乔治呼吸问卷(SGRQ)。结果 HRCT发现52例(76.5%)SSc患者存在间质性肺病(SSc-ILD),其中20例(38.5%)无呼吸道症状。最常见的HRCT表现为网格影(80.8%)、磨玻璃影(73.1%)、牵拉性支气管扩张(59.6%)和蜂窝肺(30.8%)。SSc-ILD患者中有30例(57.7%)以纤维化(网格影、蜂窝肺和牵拉性支气管扩张)为主要表现,单纯磨玻璃影仅见于8例(15.7%)。57例(83.8%)SSc患者存在弥散功能障碍,FVC和TLC下降见于28例(41.2%)和20例(29.4%)患者。肺功能指标中,DLCO与HRCT间质病变范围相关性最显著(rs=-0.476,P=0.000),并且与4项SGRQ评分均显著负相关(r=-0.392^-0.595,P<0.05)。多因素分析显示DLCO、SpO2和Borg指数是SGRQ评分的主要影响因素。结论中国SSc患者肺部受累常见且起病隐匿,诊断时HRCT表现以纤维化为主合并磨玻璃影,有必要对SSc患者进行胸部HRCT筛查。SSc肺功能损害的主要特点为弥散障碍,DLCO对于早期发现SSc-ILD以及评价严重程度具有重要价值。SGRQ可用于评价SSc患者的健康相关生活质量,并且反映了肺部受累的严重程度。

Objective To investigate the lung involvement in Chinese patients with systemic sclerosis（SSc） and its functional impact.Methods 68 patients with SSc were enrolled in Scleroderma study of PUMCH（Peking Union Medical College Hospital）.All the patients underwent high resolution computed tomography（HRCT）,pulmonary function testing,6-minute walk testing,and echocardiography.And 36 patients filled in the Saint George＇s Respiratory Questionnaire（SGRQ） for assessment of health-related quality of life.Results HRCT revealed interstitial lung disease（ILD） in 52（76.5%） patients,20 out of them without respiratory symptoms.Reticular opacification,ground-glass opacity（GGO）,traction bronchiectasis,and honeycomb were presented respectively in 80.8%,73.1%,59.6%,and 30.8% of patients with SSc-ILD.Fibrosis was the predominant HRCT finding,and pure GGO（in the absence of reticulation or architectural distortion） was only present in 8（15.7%） patients.57（83.8%） patients presented with diffusion defect,with most of them having moderate to severe impairment.Reduced FVC or TLC presented in 20（29.4%） and 28（41.2%） of patients respectively.The significant correlation was observed between the DLCO and the extent of ILD on HRCT（rs=-0.476,P=0.000）.DLCO showed significant correlations with all the four components of the SGRQ（P0.05）.Significant correlations were also observed among the SGRQ scores and SpO2（maximum desaturation） or Borg index.Stepwise multiple regression analysis confirmed that the DLCO,SpO2,and Borg index contributed to the SGRQ.Conclusions Lung involvement in Chinese SSc patients is common and insidious.The HRCT features of SSc-ILD are predominant fibrosis plus GGO,indicating little reversibility.Thus HRCT should be performed routinely in newly diagnosed SSc patients for early screening of lung involvement.The lung function defect of SSc is characterized by reduced diffusing capacity,and DLCO show better correlations with HRCT abnormities or SGRQ than FVC or TLC.Thus DLCO is of great value for early detection or severity assessment of SSc-ILD.SGRQ can be used to examine the health related quality of life of SSc patients and may reflect severity of lung involvement.