Collum Scott D, Amione-Guerra Javier, Cruz-Solbes Ana S, DiFrancesco Amara, Hernandez Adriana M, Hanmandlu Ankit, Youker Keith, Guha Ashrith, Karmouty-Quintana Harry
Department of Biochemistry and Molecular Biology, University of Texas Medical School at Houston, Houston, TX 77030, USA.
Methodist DeBakey Heart and Vascular Center, The Methodist Hospital, Houston, TX 77030, USA; Methodist J.C. Walter Jr. Transplant Center, The Methodist Hospital, Houston, TX 77030, USA.
Can Respir J. 2017;2017:1430350. doi: 10.1155/2017/1430350. Epub 2017 Feb 13.
Pulmonary hypertension (PH) is commonly present in patients with chronic lung diseases such as Chronic Obstructive Pulmonary Disease (COPD) or Idiopathic Pulmonary Fibrosis (IPF) where it is classified as Group III PH by the World Health Organization (WHO). PH has been identified to be present in as much as 40% of patients with COPD or IPF and it is considered as one of the principal predictors of mortality in patients with COPD or IPF. However, despite the prevalence and fatal consequences of PH in the setting of chronic lung diseases, there are limited therapies available for patients with Group III PH, with lung transplantation remaining as the most viable option. This highlights our need to enhance our understanding of the molecular mechanisms that lead to the development of Group III PH. In this review we have chosen to focus on the current understating of PH in IPF, we will revisit the main mediators that have been shown to play a role in the development of the disease. We will also discuss the experimental models available to study PH associated with lung fibrosis and address the role of the right ventricle in IPF. Finally we will summarize the current available treatment options for Group III PH outside of lung transplantation.
肺动脉高压（PH）常见于慢性肺病患者，如慢性阻塞性肺疾病（COPD）或特发性肺纤维化（IPF）患者，世界卫生组织（WHO）将其归类为III组PH。已确定高达40%的COPD或IPF患者存在PH，它被认为是COPD或IPF患者死亡的主要预测因素之一。然而，尽管在慢性肺病背景下PH的患病率和致命后果显著，但III组PH患者可用的治疗方法有限，肺移植仍然是最可行的选择。这凸显了我们需要加强对导致III组PH发生的分子机制的理解。在本综述中，我们选择专注于目前对IPF中PH的理解，我们将重新审视已证明在该疾病发生中起作用的主要介质。我们还将讨论可用于研究与肺纤维化相关的PH的实验模型，并探讨右心室在IPF中的作用。最后，我们将总结除肺移植外目前III组PH可用的治疗选择。
