Department of Pulmonary and Critical Care Medicine, Cleveand Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL, 33331, USA.
Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, 9500 Euclid Avenue, Desk A90, Cleveland, OH, 44195, USA.
Lung. 2017 Aug;195(4):403-409. doi: 10.1007/s00408-017-0005-6. Epub 2017 Apr 29.
Systemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. We investigated pulmonary artery dimensions as an outcome predictor in patients with SSc-ILD.
A retrospective chart review abstracting data from SSc-ILD patients evaluated at a large tertiary care center was performed. HRCT imaging was reviewed and pulmonary artery (PA) and ascending aorta (Ao) diameters were measured for calculation of the PA:Ao ratio. Additionally, demographics, vital signs, spirometric parameters, comorbidities, and mean pulmonary artery pressures were collected when available. Outcome analysis with lung transplant or death events within 4 years based on pulmonary artery size as well as PA:Ao ratio was performed.
70 SSc-ILD patients were identified. Mean pulmonary artery diameter and PA:Ao ratio was 31.17 and 1.07 mm, respectively. Patients with a pulmonary artery diameter ≥32 mm had higher risk of lung transplantation or death (p < 0.001) within 4 years. Patients with a PA:Ao ratio ≥1.1 also had higher risk of lung transplantation or death (p < 0.001) within 4 years. Unadjusted outcomes analyses also identified PA:Ao ratio ≥1.1 as an independent outcome predictor (hazard ratio 3.30, p < 0.001).
CONCLUSIONS/CLINICAL IMPLICATIONS: In SSc-ILD patients, a PA:Ao ratio ≥1.1 is associated with higher risk of lung transplant or death. These data suggest that PA:Ao dimension may be used for prognostication in SSc-ILD.
系统性硬化症是一种慢性衰弱性自身免疫性疾病，其特征为内皮功能障碍和多器官纤维化。间质性肺病是 SSc 的常见表现，被称为硬皮病相关间质性肺病（SSc-ILD），与肺动脉高压一起导致 SSc 患者的大多数死亡。SSc-ILD 患者常发生肺动脉高压，这预示着预后较差。我们研究了 SSc-ILD 患者的肺动脉尺寸作为预后预测指标。
对一家大型三级保健中心评估的 SSc-ILD 患者进行了回顾性图表审查，提取数据。对 HRCT 成像进行了审查，并测量了肺动脉（PA）和升主动脉（Ao）的直径，以计算 PA:Ao 比值。此外，收集了人口统计学数据、生命体征、肺功能参数、合并症和平均肺动脉压（当有数据时）。根据肺动脉大小以及 PA:Ao 比值进行了 4 年内肺移植或死亡事件的预后分析。
确定了 70 例 SSc-ILD 患者。平均肺动脉直径和 PA:Ao 比值分别为 31.17 和 1.07mm。肺动脉直径≥32mm 的患者在 4 年内发生肺移植或死亡的风险更高（p<0.001）。PA:Ao 比值≥1.1 的患者在 4 年内发生肺移植或死亡的风险也更高（p<0.001）。未调整的预后分析还确定 PA:Ao 比值≥1.1 是独立的预后预测指标（危险比 3.30，p<0.001）。
结论/临床意义：在 SSc-ILD 患者中，PA:Ao 比值≥1.1 与肺移植或死亡风险增加相关。这些数据表明，PA:Ao 维度可用于 SSc-ILD 的预后。
