目的：回顾性评估超声心动图估计肺动脉收缩压（sPAP）升高的发生率（IR），怀疑肺动脉高压（PH），在采用波生坦和西地那非联合治疗治疗或预防数字溃疡后的系统性硬化症（SSc）患者中。
方法：纳入2010年7月至2023年7月在Careggi大学医院硬皮病病房就诊的患者。18岁以上有数字溃疡病史的患者，波生坦和西地那非联合治疗至少12个月，包括在内。排除在引入治疗之前诊断为PH的患者。人口统计数据,疾病持续时间,实验室，和仪器数据(肺功能测试，sPAP的超声心动图估计，并收集肾阻力指数的超声值）。以事件/1000名患者年计算怀疑有肺动脉高压的超声心动图征象的IR及其95％置信区间。
结果：纳入35例患者；平均病程为12.82年（SD5.92）。联合治疗的平均持续时间为81.03（SD43.1.3）个月，总的风险时间为2674个月.两名患者（5.7％）出现了PH的超声心动图征象（sPAP50mmHg和40mmHg）；IR计算为9/1000患者-年（95％CI7.95-10.12）。在两个病人中，右心导管(RHC)排除PAH,而另一个病人拒绝接受RHC,无法确认/排除PAH。在观察时间内观察PFTs和超声心动图sPAP的稳定性。
结论：这项回顾性研究的结果表明，内皮素受体拮抗剂和磷酸二酯酶-5（PDE5）抑制剂的联合治疗可以帮助预防SSc中的PAH；需要对更多人群进行前瞻性病例对照研究，以提高该领域的知识。
OBJECTIVE: To retrospectively evaluate the incidence rate (IR) of elevated echocardiographic estimated systolic pulmonary artery pressure (sPAP), suspected for pulmonary hypertension (PH), in systemic sclerosis (SSc) patients after the introduction of a combination therapy with bosentan and sildenafil for treatment or prevention of digital ulcers.
METHODS: Patients attending the Scleroderma Unit of the Universital Hospital of Careggi from July 2010 to July 2023 were enrolled. Patients older than 18 years old with a history of digital ulcers, treated with bosentan and sildenafil in combination for at least 12 months, were included. Patients with a diagnosis of PH preceding the introduction of the therapy were excluded. Demographical data, disease duration, laboratoristic, and instrumental data (pulmonary function tests, echocardiographic estimation of sPAP, and ultrasonographic value of renal resistive index) were collected. The IR of echocardiographic signs suspected of pulmonary hypertension and their 95% confidence interval were calculated in events/1000 patients-years.
RESULTS: Thirty-five patients were enrolled; the mean disease duration was 12.82 years (SD 5.92). The mean duration of the combination treatment was 81.03 (SD 43.1.3) months, and the total at-risk time was 2674 months. Two patients (5.7%) presented echocardiographic signs of PH (sPAP 50 mmHg and 40 mmHg); the IR was calculated to be 9/1000 patients-years (95% CI 7.95-10.12). In one of the two patients, right heart catheterism (RHC) excluded PAH, while the other patient refused to undergo RHC, and PAH could not be confirmed/excluded. The stability of PFTs and echocardiographic sPAP was observed during the observation time.
CONCLUSIONS: The results of this retrospective study suggest that combination therapy with endothelin receptor antagonists and phosphodiesterase-5 (PDE5) inhibitors could help in preventing PAH in SSc; prospective
case-control studies on a larger population are needed to improve knowledge in this field.
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