PDF(Pubmed)
Abstract：
Connective tissue diseases (CTDs) can be associated with various forms of pulmonary hypertension, including pulmonary arterial hypertension (PAH), pulmonary veno-occlusive disease, pulmonary venous hypertension, interstitial lung disease-associated pulmonary hypertension, chronic thromboembolic pulmonary hypertension, and sometimes a combination of several processes. The prevalence of PAH varies among the different CTDs, with systemic sclerosis (SSc) having the highest at 8%-12%. The most recent European Society of Cardiology/European Respiratory Society guidelines recommend routine annual screening for PAH in SSc and CTDs with SSc features. As CTDs can be associated with a myriad of presentations of pulmonary hypertension, a thorough evaluation to include a right heart catheterization to clearly delineate the hemodynamic profile is essential in developing an appropriate treatment plan. Treatment strategies will depend on the predominant phenotype of pulmonary vasculopathy. In general, management approach to CTD-PAH mirrors that of idiopathic PAH. Despite this, outcomes of CTD-PAH are inferior to those of idiopathic PAH, with those of SSc-PAH being particularly poor. Reasons for this may include extrapulmonary manifestations of CTDs, including renal disease and gastrointestinal involvement, concurrent interstitial lung disease, and differences in the innate response of the right ventricle to increased pulmonary vascular resistance. Early referral for lung transplant evaluation of patients with CTD-PAH, particularly SSc-PAH, is recommended. It is hoped that in the near future, additional therapies may be added to the armamentarium of effective treatments for CTD-PAH. Ultimately, a better understanding of the pathogenesis of CTD-PAH will be required to develop targeted therapies for this morbid condition.
摘要：
结缔组织疾病(CTDs)可与各种形式的肺动脉高压,包括肺动脉高压（PAH），肺静脉闭塞性疾病，肺静脉高压,间质性肺疾病相关肺动脉高压，慢性血栓栓塞性肺动脉高压，有时是几个过程的组合。PAH的患病率在不同的CTD之间有所不同，系统性硬化症（SSc）最高，为8％-12％。最新的欧洲心脏病学会/欧洲呼吸学会指南建议每年常规筛查SSc和具有SSc特征的CTD中的PAH。由于CTD可能与无数肺动脉高压的表现有关，在制定适当的治疗计划时,必须进行全面评估,包括右心导管检查,以清楚地描绘血流动力学特征.治疗策略将取决于肺血管病变的主要表型。总的来说,CTD-PAH的管理方法反映了特发性PAH。尽管如此,CTD-PAH的结局不如特发性PAH，SSc-PAH特别差。其原因可能包括CTDs的肺外表现，包括肾脏疾病和胃肠道受累，并发间质性肺病，以及右心室对肺血管阻力增加的先天反应的差异。CTD-PAH患者肺移植的早期转诊评估，特别是SSc-PAH，是推荐的。希望在不久的将来,CTD-PAH的有效治疗方法中可能会增加其他治疗方法。最终,需要更好地了解CTD-PAH的发病机制,以开发针对这种病态的靶向治疗.
