Clinical trials on acute exacerbations of interstitial lung diseases.

IF 2.7

Expert review of respiratory medicine Pub Date : 2025-06-22 DOI:10.1080/17476348.2025.2519861

Panagiota Tsiri, Fabiola Murgolo, Bruno Crestani

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Abstract

Introduction: Acute exacerbations in interstitial lung diseases, especially idiopathic pulmonary fibrosis, remain a significant clinical challenge associated with high mortality. Previously used empirical immunosuppressive therapies, notably cyclophosphamide, have proven ineffective.

Areas covered: A literature search was conducted using the PubMed and ClinicalTrials.gov (NCT) databases for studies published up to March 2025. This review summarizes recent randomized clinical trial data on acute exacerbations, highlighting negative results for cyclophosphamide and recombinant thrombomodulin and positive preventive effects of antifibrotics such as nintedanib and pirfenidone. The evidence supporting vaccination strategies, methodological limitations in trial design, and diagnostic challenges are also discussed.

Expert opinion: Shifting clinical practice away from harmful therapies toward precision medicine approaches, utilizing biomarkers, preventive antifibrotic strategies, and novel therapeutic targets, is essential. International collaboration in clinical research is crucial to improve patient outcomes.

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肺间质性疾病急性加重的临床试验。

肺间质性疾病的急性加重，特别是特发性肺纤维化，仍然是一个与高死亡率相关的重大临床挑战。以前使用的经验性免疫抑制疗法，特别是环磷酰胺，已被证明无效。覆盖领域：使用PubMed和ClinicalTrials.gov （NCT）数据库进行文献检索，检索截至2025年3月发表的研究。这篇综述总结了最近关于急性加重的随机临床试验数据，强调了环磷酰胺和重组血栓调节素的阴性结果和抗纤维化药物如尼达尼布和吡非尼酮的阳性预防作用。支持疫苗接种策略的证据、试验设计的方法学限制和诊断挑战也进行了讨论。专家意见：将临床实践从有害疗法转向精准医学方法，利用生物标志物、预防性抗纤维化策略和新的治疗靶点是必不可少的。临床研究中的国际合作对改善患者预后至关重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Expert review of respiratory medicine

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