Lee Michael H, Bull Todd M
Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado School of Medicine, Colorado, USA.
F1000Res. 2019 Dec 19;8. doi: 10.12688/f1000research.20313.1. eCollection 2019.
Pulmonary arterial hypertension, categorized as group 1 pulmonary hypertension by the World Health Organization classification system, represents a major complication of systemic sclerosis resulting from pulmonary vascular involvement of the disease. The high mortality seen in systemic sclerosis-associated pulmonary arterial hypertension is likely due to the impairment of right ventricular systolic function and the coexistence of other non-group-1 pulmonary hypertension phenotypes that may negatively impact clinical response to pulmonary arterial hypertension-targeted therapy. This review highlights two areas of recent advances regarding the management of systemic sclerosis patients with pulmonary hypertension: the tolerability of pulmonary arterial hypertension-targeted therapy in the presence of mild to moderate interstitial lung disease and the potential clinical significance of the antifibrotic effect of soluble guanylate cyclase stimulators demonstrated in preclinical studies.
肺动脉高压，根据世界卫生组织分类系统被归类为1组肺动脉高压，是系统性硬化症的主要并发症，由该疾病的肺血管受累引起。系统性硬化症相关肺动脉高压的高死亡率可能归因于右心室收缩功能受损以及其他非1组肺动脉高压表型的共存，这些表型可能对肺动脉高压靶向治疗的临床反应产生负面影响。本综述重点介绍了系统性硬化症合并肺动脉高压患者管理方面的两个最新进展领域：在轻度至中度间质性肺病存在的情况下肺动脉高压靶向治疗的耐受性，以及临床前研究中证明的可溶性鸟苷酸环化酶刺激剂抗纤维化作用的潜在临床意义。
