Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy,
Radiol Med. 2013 Dec;118(8):1360-72. doi: 10.1007/s11547-013-0934-1. Epub 2013 May 27.
This study sought to evaluate, by means of a retrospective analysis, the relationship between pulmonary hypertension (PH) and fibrotic interstitial lung disease (ILD) in a population of 43 patients affected by systemic sclerosis. In addition, we assessed the role of high-resolution computed tomography (HRCT) in the diagnostic and therapeutic pathway for such patients.
Forty-three patients affected by progressive systemic sclerosis (PSS) and PH underwent functional, haemodynamic and HRCT evaluations between November 2001 and May 2011. Functional evaluation was performed through respiratory function testing and spirometry. Haemodynamic assessment was done with transthoracic echocardiogram (ECG) and right heart catheterisation. Semiquantitative evaluation of radiological involvement was performed using different measures: visual score, Goh's score, Wells' score and Warrick's score.
Of 43 patients, 16 showed fibrotic ILD after a HRCT evaluation. The main pattern of ILD was identified with nonspecific interstitial pneumonia (NSIP). Our study also evaluated the relationship among radiological, functional and haemodynamic indexes; different correlations were found to be significant, in particular, Wells' and visual scores ≥40% correlated significantly with diffusion capacity for carbon monoxide (DLCO); Wells' score also correlated significantly with percentage of predicted forced vital capacity (FVC), percentage of predicted forced expiratory volume in 1 s (FEV1) and composite physiologic index (CPI). If presence of Inoesophagopathy evaluated with HRCT was included, this sign correlated significantly both with DLCO (p=0.03) and mean pulmonary arterial pressure (mPAP) (p=0.03), considering total population.
Our findings confirm the diagnostic role of HRCT in evaluating the extent of lung damage in systemic sclerosis. Furthermore, a significant correlation between mPAP and esophagopathy suggests a possible positive predictive value of this sign in identifying a subphenotypic category of patients affected by systemic sclerosis in whom we can find a more severe visceral impairment and a more frequent vascular involvement.
本研究通过回顾性分析，评估了 43 例系统性硬化症患者的肺动脉高压（PH）与纤维化间质性肺疾病（ILD）之间的关系。此外，我们评估了高分辨率计算机断层扫描（HRCT）在这类患者的诊断和治疗途径中的作用。
2001 年 11 月至 2011 年 5 月期间，43 例进行性系统性硬化症（PSS）合并 PH 的患者接受了功能、血流动力学和 HRCT 评估。功能评估通过呼吸功能测试和肺活量测定进行。血流动力学评估通过经胸超声心动图（ECG）和右心导管术进行。使用不同的指标对放射学受累进行半定量评估：视觉评分、Goh 评分、Wells 评分和 Warrick 评分。
43 例患者中，16 例 HRCT 评估后显示纤维化间质性肺病。ILD 的主要模式为非特异性间质性肺炎（NSIP）。我们的研究还评估了放射学、功能和血流动力学指标之间的关系；发现不同的相关性具有显著意义，特别是 Wells 评分和视觉评分≥40%与一氧化碳弥散量（DLCO）显著相关；Wells 评分也与预计用力肺活量百分比（FVC）、预计 1 秒用力呼气量百分比（FEV1）和综合生理指数（CPI）显著相关。如果将 HRCT 评估的食管病变包括在内，考虑到总人群，该征象与 DLCO（p=0.03）和平均肺动脉压（mPAP）（p=0.03）显著相关。
我们的发现证实了 HRCT 在评估系统性硬化症患者肺部损伤程度方面的诊断作用。此外，mPAP 与食管病变之间存在显著相关性，表明该征象在识别系统性硬化症患者亚表型类别方面可能具有阳性预测价值，在这些患者中，我们可以发现更严重的内脏损害和更频繁的血管受累。
