Pulmonary hypertension (PH) due to interstitial lung disease (ILD), a commonly encountered complication of fibrotic ILDs, is associated with significant morbidity and mortality. Until recently, the studies of pulmonary vasodilator therapy in PH-ILD have been largely disappointing, with some even demonstrating the potential for harm. This paper is part of a series of Consensus Statements from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative for Group 3 Pulmonary Hypertension, with prior publications covering pathogenesis, prevalence, clinical features, phenotyping, clinical trials, and impact of PH-ILD. It offers a comprehensive review of and a holistic approach to treatment of PH-ILD, including the management of underlying interstitial lung diseases, importance of treating the comorbidities, emphasis on importance of exercise and palliation of dyspnea, and review of the most up-to-date guidelines for referral for potential lung transplant work up. It also summarizes the prior, ongoing, and possibly future studies in treatment of the vascular derangement of this morbid condition.

中文翻译：

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间质性肺疾病中的肺动脉高压：管理和治疗方法。肺血管研究所创新药物开发计划共识声明——第3组肺动脉高压

间质性肺疾病 (ILD) 引起的肺动脉高压 (PH) 是纤维化 ILD 的常见并发症，与显着的发病率和死亡率相关。直到最近，肺血管扩张剂治疗 PH-ILD 的研究大多令人失望，有些甚至证明了潜在的危害。本文是肺血管研究所第 3 类肺动脉高压创新药物开发计划一系列共识声明的一部分，之前的出版物涵盖了 PH-ILD 的发病机制、患病率、临床特征、表型、临床试验和影响。它提供了对 PH-ILD 治疗的全面回顾和整体方法，包括潜在间质性肺疾病的治疗、治疗合并症的重要性、强调运动和缓解呼吸困难的重要性，以及回顾最新的治疗方法。 - 转诊潜在肺移植检查的日期指南。它还总结了先前、正在进行的以及未来可能进行的治疗这种病态血管紊乱的研究。