Tian Lin, Wang Yun, Qi Wenlong, Wang Bingsen, Zhang Xudong, Gong Mingxue, Zhang Xiang, Wang Tan
Pulmonary Disease Tumor Blood Center, Affiliated Hospital of Changchun University of Traditional Chinese Medicine, Changchun 130021, China.
Thoracic Surgery, Affiliated Hospital of Changchun University of Traditional Chinese Medicine, Changchun 130021, China.
Biomol Ther (Seoul). 2025 Sep 1;33(5):785-803. doi: 10.4062/biomolther.2025.003. Epub 2025 Aug 14.
Interstitial lung disease (ILD) represents a heterogeneous group of diseases in which inflammation and/or fibrosis in the pulmonary interstitium results in an impaired gas exchange, difficulties in breathing, and reduced quality of daily life, and contributes to elevated global morbidity and mortality rates. ILD is an umbrella term, with idiopathic pulmonary fibrosis (IPF) being a prime focus because of its progressive and severe form. Out of 300 underlying etiologies, ILD is one of the major reasons for global morbidity and mortality. This review offers a comprehensive overview of six main categories of ILD covering autoimmune, idiopathic interstitial pneumonia, hypersensitivity pneumonitis, drug-induced, infection-related, and unclassified ILD that underscore the complexity of diagnosis and treatment challenges. This review also provides an evidence-based overview of recent advancements in the diagnosis and management of ILD, with precision pharmacotherapy, multidisciplinary care, and emerging therapeutic strategies. From clinical trial data, it also recommends the disease-specific use of pharmacological agents-such as pirfenidone and nintedanib for IPF, and mycophenolate mofetil for connective tissue disease-associated ILD. The manuscript also emphasizes the evolving role of non-pharmacological interventions, including the 6-minute walk test and pulmonary rehabilitation, in enhancing functional capacity and quality of life. To address the current global health concerns, topics of post-COVID-19 ILD and immune checkpoint inhibitor-associated lung disease are integrated. Additionally, future directions are explored, including the role of lung transplantation and novel antifibrotic therapies like anti-Transforming Growth Factor (TGF)-β antibody cocktails. Together, these insights aim to refine diagnostic precision, personalize treatment, and improve clinical outcomes across the heterogeneous ILD spectrum.
间质性肺疾病（ILD）是一组异质性疾病，其中肺间质的炎症和/或纤维化会导致气体交换受损、呼吸困难以及日常生活质量下降，并导致全球发病率和死亡率升高。ILD是一个统称，特发性肺纤维化（IPF）因其进展性和严重形式而成为主要关注对象。在300种潜在病因中，ILD是全球发病和死亡的主要原因之一。本综述全面概述了ILD的六大主要类别，包括自身免疫性、特发性间质性肺炎、过敏性肺炎、药物性、感染相关性和未分类的ILD，突出了诊断和治疗挑战的复杂性。本综述还基于证据概述了ILD诊断和管理的最新进展，包括精准药物治疗、多学科护理和新兴治疗策略。从临床试验数据来看，它还推荐了针对特定疾病使用的药物，如用于IPF的吡非尼酮和尼达尼布，以及用于结缔组织病相关ILD的霉酚酸酯。该手稿还强调了非药物干预措施，包括6分钟步行试验和肺康复，在提高功能能力和生活质量方面不断演变的作用。为解决当前全球健康问题，纳入了新冠病毒感染后ILD和免疫检查点抑制剂相关肺部疾病的主题。此外，还探讨了未来的方向，包括肺移植的作用以及新型抗纤维化疗法，如抗转化生长因子（TGF）-β抗体鸡尾酒疗法。总之，这些见解旨在提高诊断精度、实现个性化治疗并改善整个异质性ILD谱系的临床结果。
