BACKGROUND There are limited data assessing the spectrum of systemic sclerosis-associated pulmonary hypertension (PH). METHODS Data for 912 systemic sclerosis patients assessed between 2000 and 2020 were retrieved from the Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre (ASPIRE) registry and classified based on 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines and multimodality investigations. RESULTS Reduction in pulmonary vascular resistance (PVR) diagnostic threshold to >2WU resulted in a 19% increase in precapillary PH diagnoses. Patients with PVR ≤2WU had superior survival to PVR >2-3WU which was similar to PVR >3-4WU. Survival in pulmonary arterial hypertension (PAH) was superior to PH associated with lung disease. However, patients with mild parenchymal disease on CT had similar characteristics and outcomes to patients without lung disease. Combined pre- and postcapillary PH had significantly poorer survival than isolated postcapillary PH. Patients with mean pulmonary arterial wedge pressure (PAWP) 13-15 mm Hg had similar haemodynamics and left atrial volumes to those with PAWP >15 mm Hg. Unclassified-PH had more frequently dilated left atria and higher PAWP than PAH. Although Unclassified-PH had a similar survival to No-PH, 36% were subsequently diagnosed with PAH or PH associated with left heart disease. The presence of 2-3 radiological signs of pulmonary veno-occlusive disease was noted in 7% of PAH patients and was associated with worse survival. Improvement in incremental shuttle walking distance of ≥30 m following initiation of PAH therapy was associated with superior survival. PAH patients diagnosed after 2011 had greater use of combination therapy and superior survival. CONCLUSION A number of systemic sclerosis PH phenotypes can be recognized and characterized using haemodynamics, lung function and multimodality imaging.

中文翻译：

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系统性硬化症相关肺动脉高压的范围：来自 ASPIRE 登记处的见解。


背景 评估系统性硬化症相关肺动脉高压 （PH） 谱的数据有限。方法 从 2000 年至 2020 年间评估的 912 例系统性硬化症患者的数据检索在研究中心 （ASPIRE） 登记处确定的肺动脉高压谱，并根据 2022 年欧洲心脏病学会/欧洲呼吸学会 （ESC/ERS） 指南和多模式调查进行分类。结果 肺血管阻力 （PVR） 诊断阈值降低至 >2WU 导致毛细血管前 PH 诊断增加 19%。PVR ≤2WU 患者的生存率优于 PVR >2-3WU，后者与 PVR >3-4WU 相似。肺动脉高压 （PAH） 的生存率优于与肺部疾病相关的 PH。然而，CT 上轻度实质疾病患者与无肺病患者具有相似的特征和结局。平均肺动脉楔压 （PAWP） 为 13-15 mm Hg 的患者与 PAWP >15 mm Hg 的患者具有相似的血流动力学和左心房容积。未分类 PH 的左心房扩张更频繁，PAWP 高于 PAH。尽管未分类 PH 的生存率与无 PH 相似，但 36% 的患者随后被诊断出患有 PAH 或与左心疾病相关的 PH。在 7% 的 PAH 患者中观察到 2-3 种肺静脉闭塞性疾病的放射学体征，并且与较差的生存率相关。PAH 治疗开始后 ≥30 m 的增量穿梭步行距离改善与优越的生存率相关。2011 年之后诊断的 PAH 患者更多地使用联合治疗和更高的生存率。 结论 使用血流动力学、肺功能和多模态成像可以识别和表征许多系统性硬化症 PH 表型。