We devised a scoring system to identify patients with systemic sclerosis (SSc) at risk for pulmonary hypertension (PH) and predict all-cause mortality. Using 7 variables obtained via pulmonary function testing, echocardiography, and computed tomographic chest imaging, we applied the score to a retrospective cohort of 117 patients with SSc. There were 60 (51.3%) who were diagnosed with PH by right heart catheterization. Using a scoring threshold ≥ 0, our decision tool predicted PH with a sensitivity, specificity, and accuracy of 0.87 (95% CI 0.75, 0.94), 0.74 (95% CI 0.60, 0.84), and 0.80 (95% CI 0.72, 0.87), respectively. When adjusted for age at PH diagnosis, sex, and receipt of pulmonary arterial vasodilators, each one-point score increase was associated with an adjusted HR of 1.19 (95% CI 1.05, 1.34) for all-cause mortality. With further validation in external cohorts, our simplified clinical decision tool may better streamline earlier detection of PH in SSc.

我们设计了一个评分系统来识别有肺动脉高压（PH）风险的系统性硬化症（SSc）患者并预测全因死亡率。使用通过肺功能测试、超声心动图和计算机断层胸部成像获得的 7 个变量，我们将评分应用于 117 名 SSc 患者的回顾性队列。经右心导管检查诊断为PH的有60人（51.3%）。使用评分阈值 ≥ 0，我们的决策工具预测 PH 的敏感性、特异性和准确性分别为 0.87 (95% CI 0.75, 0.94)、0.74 (95% CI 0.60, 0.84) 和 0.80 (95% CI 0.72, 0.87) ）， 分别。当根据 PH 诊断时的年龄、性别和接受肺动脉血管扩张剂进行调整时，每增加 1 分的分数与调整后的全因死亡率 HR 为 1.19（95% CI 1.05，1.34）相关。通过外部队列的进一步验证，我们简化的临床决策工具可以更好地简化 SSc 中 PH 的早期检测。