Institute of Clinical Physiology, National Research Council, Via Moruzzi, 1, Pisa 56124, Italy.
Department of Cardiology, University Hospital of Nancy, Institut Lorrain du Cœur et des Vaisseaux, 5 Rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.
Heart Fail Clin. 2018 Jul;14(3):271-281. doi: 10.1016/j.hfc.2018.02.004.
Involvement of the right heart-pulmonary circulation system in systemic sclerosis is a typical feature, with critical prognostic implications. Pulmonary hypertension may occur in association with interstitial lung disease or as a result of an isolated pulmonary vascular disease that may affect both the precapillary arterioles and the postcapillary venules, as well as a consequence of left heart involvement. These apparently different phenotypes often underlie a significant pathophysiologic overlap, which makes the diagnosis and management of these patients highly complex and uncertain.
系统性硬化症中右心-肺循环系统的受累是一个典型特征，具有重要的预后意义。肺动脉高压可能与间质性肺疾病相关，也可能是由于孤立性肺血管疾病引起，这种疾病可能影响毛细血管前小动脉和毛细血管后小静脉，也可能是由于左心受累引起。这些明显不同的表型通常存在显著的病理生理学重叠，这使得这些患者的诊断和管理变得非常复杂和不确定。
