Dietrich Jana, Kang Alice, Tielemans Birger, Verleden Stijn E, Khalil Hassan, Länger Florian, Bruners Philipp, Mentzer Steven J, Welte Tobias, Dreher Michael, Jonigk Danny D, Ackermann Maximilian
Institute of Pathology, University Clinics Aachen, RWTH University of Aachen, Aachen, Germany.
J. Dietrich and A. Kang share first authorship.
Eur Respir Rev. 2025 Feb 5;34(175). doi: 10.1183/16000617.0080-2024. Print 2025 Jan.
Interstitial lung disease (ILD) is a clinical term that refers to a diverse group of non-neoplastic lung diseases. This group includes idiopathic and secondary pulmonary entities that are often associated with progressive pulmonary fibrosis. Currently, therapeutic approaches based on specific structural targeting of pulmonary fibrosis are limited to nintedanib and pirfenidone, which can only slow down disease progression leading to a lower mortality rate. Lung transplantation is currently the only available curative treatment, but it is associated with high perioperative mortality. The pulmonary vasculature plays a central role in physiological lung function, and vascular remodelling is considered a hallmark of the initiation and progression of pulmonary fibrosis. Different patterns of pulmonary fibrosis commonly exhibit detectable pathological features such as morphomolecular changes, including intussusceptive and sprouting angiogenesis, vascular morphometry, broncho-systemic anastomoses, and aberrant angiogenesis-related gene expression patterns. Dynamic cellular interactions within the fibrovascular interface, such as endothelial activation and endothelial-mesenchymal transition, are also observed. This review aims to summarise the current clinical, radiological and pathological diagnostic algorithm for different ILDs, including usual interstitial pneumonia/idiopathic pulmonary fibrosis, non-specific interstitial pneumonia, alveolar fibroelastosis/pleuroparenchymal fibroelastosis, hypersensitivity pneumonitis, systemic sclerosis-related ILD and coronavirus disease 2019 injury. It emphasises an interdisciplinary clinicopathological perspective. Additionally, the review covers current therapeutic strategies and knowledge about associated vascular abnormalities.
间质性肺疾病（ILD）是一个临床术语，指的是一组多样的非肿瘤性肺疾病。这组疾病包括特发性和继发性肺部疾病，它们通常与进行性肺纤维化相关。目前，基于肺纤维化特定结构靶点的治疗方法仅限于尼达尼布和吡非尼酮，它们只能减缓疾病进展，降低死亡率。肺移植是目前唯一可用的治愈性治疗方法，但它与围手术期高死亡率相关。肺血管系统在肺的生理功能中起核心作用，血管重塑被认为是肺纤维化发生和进展的标志。不同类型的肺纤维化通常表现出可检测到的病理特征，如形态分子变化，包括套叠式和芽生式血管生成、血管形态测定、支气管-体循环吻合以及异常的血管生成相关基因表达模式。在纤维血管界面还观察到动态的细胞相互作用，如内皮细胞活化和内皮-间充质转化。本综述旨在总结不同ILD的当前临床、放射学和病理诊断算法，包括寻常型间质性肺炎/特发性肺纤维化、非特异性间质性肺炎、肺泡纤维弹性组织增生症/胸膜肺实质纤维弹性组织增生症（pleuroparenchymal fibroelastosis）、过敏性肺炎、系统性硬化症相关ILD和2019冠状病毒病损伤。它强调跨学科的临床病理视角。此外，该综述涵盖了当前的治疗策略以及关于相关血管异常的知识。
