Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Curr Opin Pulm Med. 2023 Sep 1;29(5):380-390. doi: 10.1097/MCP.0000000000001001. Epub 2023 Jul 14.
Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc), which confers significant morbidity and mortality. The current therapies and treatment strategies for SSc-associated PAH (SSc-PAH) are informed by those used to treat patients with idiopathic PAH (IPAH). There are, however, important differences between these two diseases that impact diagnosis, treatment, and outcomes.
Both SSc-PAH and IPAH are incompletely understood with ongoing research into the underlying cellular biology that characterize and differentiate the two diseases. Additional research seeks to improve identification among SSc patients in order to diagnose patients earlier in the course of their disease. Novel therapies specifically for SSc-PAH such as rituximab and dimethyl fumarate are under investigation.
Although patients with SSc-PAH and IPAH present with similar symptoms, there are significant differences between these two forms of PAH that warrant further investigation and characterization of optimal detection strategies, treatment algorithms, and outcomes assessment.
肺动脉高压（PAH）是系统性硬化症（SSc）的常见并发症，会导致较高的发病率和死亡率。目前用于治疗特发性 PAH（IPAH）的治疗方法和治疗策略同样适用于 SSc 相关 PAH（SSc-PAH）。然而，这两种疾病之间存在重要差异，这些差异会影响诊断、治疗和预后。
SSc-PAH 和 IPAH 的发病机制均尚未完全明确，目前正在进行基础细胞生物学研究，以进一步阐明和区分这两种疾病。此外，研究旨在改进 SSc 患者的识别方法，以便在疾病早期对患者进行诊断。利妥昔单抗和二甲基富马酸等专门针对 SSc-PAH 的新型疗法正在研究中。
虽然 SSc-PAH 和 IPAH 患者表现出相似的症状，但这两种 PAH 形式存在显著差异，需要进一步研究和确定最佳检测策略、治疗方案和预后评估。
