Lung transplantation (LTx) can be a life-extending treatment option for patients with advanced and/or progressive fibrotic interstitial lung disease (ILD), especially idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis, sarcoidosis and connective tissue disease-associated ILD. IPF is now the most common indication for LTx worldwide. Several unique features in patients with ILD can impact optimal timing of referral or listing for LTx, pre- or post-transplant risks, candidacy and post-transplant management. As the epidemiology of LTx and community practices have evolved, recent literature describes outcomes and approaches in higher-risk candidates. In this review, we discuss the unique and important clinical findings, course, monitoring and management of patients with IPF and other progressive fibrotic ILDs during pre-LTx evaluation and up to the day of transplantation; the need for co-management with clinical experts in ILD and LTx is emphasised. Some post-LTx complications are unique in these patient cohorts, which require prompt detection and appropriate management by experts in multiple disciplines familiar with telomere biology disorders and infectious, haematological, oncological and cardiac complications to enhance the likelihood of improved outcomes and survival of LTx recipients with IPF and other ILDs.

肺移植 (LTx) 可以成为晚期和/或进行性纤维化间质性肺病 (ILD) 患者延长生命的治疗选择，尤其是特发性肺纤维化 (IPF)、纤维化过敏性肺炎、结节病和结缔组织病相关 ILD。IPF 现在是全球 LTx 最常见的适应症。ILD 患者的几个独特特征会影响 LTx 转诊或上市的最佳时机、移植前或移植后风险、候选资格和移植后管理。随着 LTx 的流行病学和社区实践的发展，最近的文献描述了高风险候选人的结果和方法。在这篇综述中，我们讨论了独特而重要的临床发现，当然，在 LTx 前评估期间和直至移植当天监测和管理 IPF 和其他进行性纤维化 ILD 患者；强调需要与 ILD 和 LTx 临床专家共同管理。一些 LTx 后并发症在这些患者队列中是独一无二的，需要由熟悉端粒生物学障碍和感染、血液学、肿瘤学和心脏并发症的多学科专家及时检测和适当管理，以提高 LTx 接受者改善结果和生存的可能性与 IPF 和其他 ILD。