Mathai Stephen C
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, 1830 E. Monument Street, Room 540, Baltimore, MD 21205, USA.
Rheum Dis Clin North Am. 2024 Aug;50(3):359-379. doi: 10.1016/j.rdc.2024.03.005.
Pulmonary hypertension (PH), a syndrome characterized by elevated pulmonary pressures, commonly complicates connective tissue disease (CTD) and is associated with increased morbidity and mortality. The incidence of PH varies widely between CTDs; patients with systemic sclerosis are most likely to develop PH. Several different types of PH can present in CTD, including PH related to left heart disease and respiratory disease. Importantly, CTD patients are at risk for developing pulmonary arterial hypertension, a rare form of PH that is associated with high morbidity and mortality. Future therapies targeting pulmonary vascular remodeling may improve outcomes for patients with this devastating disease.
肺动脉高压（PH）是一种以肺动脉压力升高为特征的综合征，常使结缔组织病（CTD）复杂化，并与发病率和死亡率增加相关。PH在CTD中的发病率差异很大；系统性硬化症患者最易发生PH。CTD可出现几种不同类型的PH，包括与左心疾病和呼吸系统疾病相关的PH。重要的是，CTD患者有发生肺动脉高压的风险，肺动脉高压是一种罕见的PH形式，与高发病率和死亡率相关。针对肺血管重塑的未来疗法可能会改善患有这种毁灭性疾病患者的预后。
