Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, 060-8638, Japan.
Department of Diagnostic Imaging, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Mod Rheumatol. 2023 Jul 4;33(4):758-767. doi: 10.1093/mr/roac102.
Systemic sclerosis (SSc) is associated with pulmonary vascular disease and interstitial lung disease, making it difficult to differentiate pulmonary arterial hypertension and pulmonary hypertension (PH) due to lung diseases and/or hypoxia and to decide treatments. We aimed to predict the response to pulmonary vasodilators in patients with SSc and PH.
Eighty-four SSc patients were included with 47 having PH. Chest computed tomography was evaluated using software to calculate the abnormal lung volume (ALV). To define the response to vasodilators, Δ mean pulmonary artery pressure (mPAP)/basal mPAP was used (cut-off value: 10%). The predictive value was evaluated by using the receiver operating characteristic curve.
The mean (±standard deviation) value of ALV was 26.8 (±32.2) %. A weak correlation was observed between ALV and forced vital capacity (FVC) (R = -0.46). The predictive value of ALV [area under curve (AUC) = 0.74] was superior to that of FVC (AUC = 0.62) for the response to vasodilators. No hemodynamic parameters differed between patients with high and low ALV, whereas survival was worse in high ALV.
Quantitative chest computed tomography well predicted the response to vasodilators in patients with SSc and PH. Our results suggest its utility in differentiating the dominance of pulmonary vascular disease or interstitial lung disease.
系统性硬化症（SSc）与肺血管疾病和间质性肺病有关，这使得难以区分肺动脉高压和由肺部疾病和/或缺氧引起的高血压（PH），并难以决定治疗方法。我们旨在预测 SSc 和 PH 患者对肺血管扩张剂的反应。
共纳入 84 例 SSc 患者，其中 47 例患有 PH。使用软件评估胸部计算机断层扫描以计算异常肺容积（ALV）。为了定义对血管扩张剂的反应，使用 Δ平均肺动脉压（mPAP）/基础 mPAP（截断值：10%）。通过使用受试者工作特征曲线评估预测值。
ALV 的平均值（±标准差）为 26.8（±32.2）%。ALV 与用力肺活量（FVC）之间存在弱相关性（R=-0.46）。ALV 的预测价值（AUC=0.74）优于 FVC（AUC=0.62）对血管扩张剂反应的预测价值。高 ALV 组和低 ALV 组的血流动力学参数无差异，而高 ALV 组的生存率较差。
定量胸部计算机断层扫描很好地预测了 SSc 和 PH 患者对血管扩张剂的反应。我们的结果表明，它可用于区分肺血管疾病或间质性肺病的优势。
