Boutel Maria, Dara Athanasia, Arvanitaki Alexandra, Deuteraiou Cleopatra, Mytilinaiou Maria, Dimitroulas Theodoros
Fourth Department of Internal Medicine, Hippokration University Hospital, School of Medicine, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece.
Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy's and St Thomas's NHS Foundation Trust, Imperial College, London SW3 6NP, UK.
J Clin Med. 2024 Sep 30;13(19):5834. doi: 10.3390/jcm13195834.
Precapillary pulmonary hypertension (PH) is a significant complication of systemic sclerosis (SSc). It represents one of the leading causes of morbidity and mortality, correlating with a significantly dismal prognosis and quality of life. Despite advancements in the management of patients with pulmonary arterial hypertension associated with SSc (SSc-PAH), no significant improvement has been reported in survival of patients with precapillary SSc-PH associated with extensive lung parenchyma disease. International expert consensus and guidelines for the management of PH recommend annual screening of SSc patients for early detection of pre-capillary PH. The implementation of screening algorithms capable of identifying patients with a high likelihood of developing PH could help limit unnecessary right-heart catheterization procedures and prevent significant delay in diagnosis. Furthermore, early initiation of up-front combination targeted therapy in patients with PAH has shown increase in survival rates, indicating that timely and aggressive medical therapy is key for stabilizing and even improving functional class, hemodynamic parameters and 6 min walking distance (6MWD) in this population. Further research is warranted into the benefit of PAH-targeted therapies in patients with PH associated with lung disease. Lastly, we discuss the potential role of immunosuppression using biologic agents in the therapeutic management of precapillary PH in SSc patients.
毛细血管前性肺动脉高压（PH）是系统性硬化症（SSc）的一种重要并发症。它是发病和死亡的主要原因之一，与显著不良的预后和生活质量相关。尽管在与SSc相关的肺动脉高压（SSc-PAH）患者的管理方面取得了进展，但对于伴有广泛肺实质疾病的毛细血管前性SSc-PH患者的生存率，尚未有显著改善的报道。国际专家关于PH管理的共识和指南建议每年对SSc患者进行筛查，以早期发现毛细血管前性PH。实施能够识别有发生PH高可能性患者的筛查算法，有助于限制不必要的右心导管检查程序，并防止诊断出现重大延误。此外，对PAH患者早期启动预先联合靶向治疗已显示生存率有所提高，这表明及时且积极的药物治疗是稳定甚至改善该人群功能分级、血流动力学参数和6分钟步行距离（6MWD）的关键。有必要进一步研究PAH靶向治疗对伴有肺部疾病的PH患者的益处。最后，我们讨论了使用生物制剂进行免疫抑制在SSc患者毛细血管前性PH治疗管理中的潜在作用。
