华中科技大学学报(医学版) ›› 2026, Vol. 55 ›› Issue (5): 742-751.doi: 10.3870/j.issn.1672-0741.25.11.006

• 综述 • 上一篇    下一篇

进展性肺纤维化的研究进展*

陈丹妮, 向菲△   

  1. 华中科技大学同济医学院附属协和医院呼吸与危重症医学科,武汉 430021
  • 收稿日期:2025-11-06 发布日期:2026-10-30
  • 通讯作者: △E-mail:2006XH0855@hust.edu.cn
  • 作者简介:陈丹妮,女,2000年生,硕士研究生,E-mail:chendn0012@163.com
  • 基金资助:
    *国家自然科学基金资助项目(No. 81300047)

Research Progress in Progressive Pulmonary Fibrosis

Chen Danni, Xiang Fei△   

  1. Department of Respiratory and Critical Care Medicine, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430021,China
  • Received:2025-11-06 Published:2026-10-30
  • Contact: △E-mail:2006XH0855@hust.edu.cn

摘要: 进展性肺纤维化(progressive pulmonary fibrosis,PPF)是指一组表现为进行性恶化表型的间质性肺疾病(interstitial lung disease,ILD),预后极差。其基于症状、肺功能及影像学进行综合判定,不局限于特定病因,而是涵盖除特发性肺纤维化外所有表现为进行性纤维化的ILD患者。目前识别具有进展风险的早期患者并进行及时干预仍存在困难,现有抗纤维化治疗仅能减缓病情进展而非逆转疾病,且部分患者耐受性不佳。基于此,该文对PPF的发病机制、临床识别、治疗方式等进行综述,旨在为未来PPF患者的早期预测及精准靶向治疗提供新的见解。

关键词: 进展性肺纤维化, 发病机制, 早期识别, 抗纤维化治疗

Abstract: Progressive pulmonary fibrosis (PPF) is a group of interstitial lung diseases (ILD) characterized by a progressive phenotype and poor prognosis. It is based on a comprehensive assessment of symptoms, lung function and imaging findings. PPF is not limited to specific etiologies, but encompasses patients with ILD presenting with progressive fibrosis, with the exception of idiopathic pulmonary fibrosis.Currently, it remains difficult to identify early-stage patients at risk of progression and provide timely intervention. Existing antifibrotic treatments only slow disease progression without reversing it, and some patients exhibit poor tolerability. Therefore, this article reviews the pathogenesis, clinical identification, and treatment strategies for PPF, aiming to provide new insights into early prediction and precision-targeted therapy for patients with PPF.

Key words: progressive pulmonary fibrosis, pathogenesis, early identification, antifibrotic treatment

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