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Pathophysiological Insights and Clinical Management Strategies for Interstitial Lung Diseases.

作者信息

Tian Lin, Wang Yun, Qi Wenlong, Wang Bingsen, Zhang Xudong, Gong Mingxue, Zhang Xiang, Wang Tan

机构信息

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.

出版信息

Biomol Ther (Seoul). 2025 Sep 1;33(5):785-803. doi: 10.4062/biomolther.2025.003. Epub 2025 Aug 14.


DOI:10.4062/biomolther.2025.003
PMID:40803754
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12408203/
Abstract

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.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/07a34a3568ef/bt-33-5-785-f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/97ecc548e215/bt-33-5-785-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/f313467a47bc/bt-33-5-785-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/adffb85ba330/bt-33-5-785-f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/07a34a3568ef/bt-33-5-785-f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/97ecc548e215/bt-33-5-785-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/f313467a47bc/bt-33-5-785-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/adffb85ba330/bt-33-5-785-f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0ffe/12408203/07a34a3568ef/bt-33-5-785-f4.jpg

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Pathophysiological Insights and Clinical Management Strategies for Interstitial Lung Diseases.

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本文引用的文献

[1]
Interstitial lung disease: a review of classification, etiology, epidemiology, clinical diagnosis, pharmacological and non-pharmacological treatment.

Front Med (Lausanne). 2024-4-18

[2]
Interstitial Lung Disease: A Review.

JAMA. 2024-5-21

[3]
Multidisciplinary Approach to the Diagnosis of Idiopathic Interstitial Pneumonias: Focus on the Pathologist's Key Role.

Int J Mol Sci. 2024-3-23

[4]
The Beneficial Impact of Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis: A Review of the Current Literature.

J Clin Med. 2024-3-30

[5]
Novel inhalation therapy in pulmonary fibrosis: principles, applications and prospects.

J Nanobiotechnology. 2024-3-29

[6]
Inhaled treprostinil in patients with pulmonary hypertension associated with interstitial lung disease with less severe haemodynamics: a post hoc analysis of the INCREASE study.

BMJ Open Respir Res. 2024-3-22

[7]
Overview of Rheumatoid Arthritis-Associated Interstitial Lung Disease and Its Treatment.

Semin Respir Crit Care Med. 2024-6

[8]
Nalbuphine Tablets for Cough in Patients with Idiopathic Pulmonary Fibrosis.

NEJM Evid. 2023-8

[9]
Safety and efficacy of rituximab in systemic sclerosis (DESIRES): a double-blind, investigator-initiated, randomised, placebo-controlled trial.

Lancet Rheumatol. 2021-7

[10]
Morphine for treatment of cough in idiopathic pulmonary fibrosis (PACIFY COUGH): a prospective, multicentre, randomised, double-blind, placebo-controlled, two-way crossover trial.

Lancet Respir Med. 2024-4

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