Pulmonary hypertension (PH) associated with chronic lung disease (CLD) is both common and underrecognised. The presence of PH in the setting of lung disease has been consistently shown to be associated with worse outcomes. Recent epidemiological studies have advanced understanding of the heterogeneity of this patient population and shown that defining both the specific type of CLD as well as the severity of PH (i.e. deeper phenotyping) is necessary to inform natural history and prognosis. A systematic diagnostic approach to screening and confirmation of suspected PH in CLD is recommended. Numerous uncontrolled studies and one phase 3 randomised, controlled trial have suggested a benefit in treating PH in some patients with CLD, specifically those with fibrotic interstitial lung disease (ILD). However, other studies in diseases such as COPD-PH showed adverse outcomes with some therapies. Given the expanding list of approved pharmacological treatments for pulmonary arterial hypertension, developing a treatment algorithm for specific phenotypes of CLD-PH is required. This article will summarise existing data in COPD, ILD and other chronic lung diseases, and provide recommendations for classification of CLD-PH and approach to the diagnosis and management of these challenging patients.

与慢性肺病 （CLD） 相关的肺动脉高压 （PH） 既常见又未得到充分认识。在肺部疾病的情况下，PH 的存在一直被证明与较差的结局相关。最近的流行病学研究加深了对该患者群体异质性的理解，并表明定义 CLD 的特定类型以及 PH 的严重程度（即 更深的表型）对于告知自然病史和预后是必要的。建议采用系统诊断方法来筛查和确认 CLD 中的疑似 PH。许多非对照研究和一项 3 期随机对照试验表明，治疗一些 CLD 患者的 PH 是有益的，特别是纤维化间质性肺病 （ILD） 患者。然而，其他针对 COPD-PH 等疾病的研究显示，某些疗法的不良结局。鉴于已批准的肺动脉高压药物治疗清单不断扩大，需要针对 CLD-PH 的特定表型开发治疗算法。本文将总结 COPD、ILD 和其他慢性肺病的现有数据，并为 CLD-PH 的分类以及这些具有挑战性的患者的诊断和管理方法提供建议。