Longitudinal Risk Phenotyping in Incident Systemic Sclerosis-associated Pulmonary Arterial Hypertension (SSc-PAH): An Unsupervised Cluster Analysis of the PHAROS Registry

Friedman Samuel H, Williams Jacob, Hyer Madison, Fox Nicholas, Ramakrishnan Viswanathan, Lammi Matthew R, Steen Virginia D, Argula Rahul G
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Abstract

Background: Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is characterized by pulmonary arterial remodeling resulting in right ventricular failure and death if untreated. Despite therapeutic advances, there is survival variability within the SSc-PAH population. The aim of this study was to delineate high-risk subgroups of SSc-PAH using readily available clinical parameters. Methods: We analyzed data from the PHAROS database, a prospective observational registry of incident SSc-PAH patients. Latent class modeling was performed based on trends in 6MWD over time. We compared survival between the clusters regarding baseline clinical parameters and changes in these parameters over time. Results: We identified four unique groups within 103 patients meeting our inclusion criteria, based on trajectories of 6MWD. Patients in Cluster 4 exhibited a decline in 6MWD over time and had the worst prognosis with a median survival of 3 years. Patients in Cluster 3, with the lowest baseline 6MWD, were associated with lower median survival (5 years) when compared to Clusters 1 and 2 (> 9 and 7 years, respectively), despite an improvement in 6MWD over time. There were no meaningful changes in SF-36 and WHO functional class between the clusters, but BNP trended higher over time in the higher-risk clusters. Conclusion: We identified high-risk subsets of SSc-PAH characterized by significantly worse survival. Incident SSc-PAH patients with a decline in 6MWD over time or low baseline 6MWD had worse survival when compared to SSc-PAH patients who demonstrated relatively stable or mild reduction in 6MWD over time.
系统性硬化症相关肺动脉高压(SSc-PAH)发病的纵向风险分型:对 PHAROS 登记的无监督聚类分析
背景:系统性硬化症相关性肺动脉高压(SSc-PAH)的特点是肺动脉重塑,如不及时治疗会导致右心室衰竭和死亡。尽管治疗取得了进展,但 SSc-PAH 患者的存活率仍存在差异。本研究旨在利用现成的临床参数来划分 SSc-PAH 的高风险亚组。方法:我们分析了来自 PHAROS 数据库的数据,该数据库是对 SSc-PAH 患者的前瞻性观察登记。根据 6MWD 随时间变化的趋势进行了潜类建模。我们比较了基线临床参数和这些参数随时间变化的群组间生存率。结果:根据 6MWD 的变化轨迹,我们在 103 名符合纳入标准的患者中确定了四个独特的群组。第 4 组患者的 6MWD 随时间推移而下降,预后最差,中位生存期为 3 年。第 3 组患者的基线 6MWD 最低,与第 1 组和第 2 组(分别大于 9 年和 7 年)相比,中位生存期(5 年)较低,尽管随着时间的推移 6MWD 有所改善。各组群之间的 SF-36 和 WHO 功能分级没有明显变化,但随着时间的推移,高风险组群的 BNP 呈上升趋势。结论:我们发现了SSc-PAH的高风险亚群,其特点是生存率明显降低。6MWD随时间下降或基线6MWD较低的SSc-PAH患者与6MWD随时间相对稳定或轻度下降的SSc-PAH患者相比,生存率更低。
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