Center for Pulmonary Heart Disease, Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
Unità di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, MultiMedica IRCCS, Milan, Italy.
Eur Respir Rev. 2022 Jul 12;31(165). doi: 10.1183/16000617.0220-2021. Print 2022 Sep 30.
Pulmonary hypertension (PH) is known to complicate various forms of interstitial lung disease (ILD), including idiopathic pulmonary fibrosis, the interstitial pneumonias and chronic hypersensitivity pneumonitis. Pathogenesis of PH-ILD remains incompletely understood, and probably has overlap with other forms of pre-capillary pulmonary hypertension. PH-ILD carries a poor prognosis, and is associated with increased oxygen requirements, and a decline in functional capacity and exercise tolerance. Despite most patients having mild-moderate pulmonary hypertension, more severe pulmonary hypertension and signs of right heart failure are observed in a subset of cases. Clinical suspicion and findings on pulmonary function, computed tomography and echocardiography are often the initial steps towards diagnosis. Definitive diagnosis is obtained by right heart catheterisation demonstrating pre-capillary pulmonary hypertension. Drugs approved for pulmonary arterial hypertension have been investigated in several randomised controlled trials in PH-ILD patients, leading to discouraging results until the recent INCREASE study. This review provides an overview of the current understanding, approach to diagnosis and recent advances in treatment.
肺动脉高压（PH）是各种类型的间质性肺疾病（ILD）的并发症，包括特发性肺纤维化、间质性肺炎和慢性过敏性肺炎。PH-ILD 的发病机制仍不完全清楚，可能与其他形式的毛细血管前性肺动脉高压重叠。PH-ILD 预后不良，与氧气需求增加、功能能力和运动耐量下降有关。尽管大多数患者有轻度-中度肺动脉高压，但在一部分病例中观察到更严重的肺动脉高压和右心衰竭的迹象。临床怀疑和肺功能、计算机断层扫描和超声心动图的发现通常是诊断的初始步骤。通过右心导管检查显示毛细血管前性肺动脉高压可获得明确诊断。已经在 PH-ILD 患者的几项随机对照试验中研究了批准用于肺动脉高压的药物，但直到最近的 INCREASE 研究才得出令人沮丧的结果。这篇综述提供了对目前认识、诊断方法和治疗进展的概述。
