特发性肺纤维化：当前和未来的治疗。,Clinical Respiratory Journal

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特发性肺纤维化：当前和未来的治疗。
Clinical Respiratory Journal ( IF 1.9 ) Pub Date : 2022-01-10 , DOI: 10.1111/crj.13466
Daniel S Glass ₁ , David Grossfeld ₁ , Heather A Renna ₁ , Priya Agarwala ₁ , Peter Spiegler ₁ , Joshua DeLeon ₁ , Allison B Reiss ₁

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目的特发性肺纤维化 (IPF) 是一种以干咳、疲劳和进行性劳力性呼吸困难为特征的慢性纤维化肺病。在这种使人衰弱的情况下，肺实质和结构被破坏，顺应性丧失，气体交换受到损害，在诊断后 3-5 年内不可避免地导致呼吸衰竭和死亡。这篇综述讨论了当前使用的 IPF 治疗方法以及那些看起来对未来发展有希望的方法。数据来源数据来自英国文献中发表的随机对照试验和科学研究。我们使用了与 IPF、抗纤维化治疗、肺移植和管理相关的搜索词。结果 IPF 的发病机制尚不完全清楚，治疗选择有限。IPF 的病理特征包括细胞外基质重塑、成纤维细胞活化和增殖、免疫失调、细胞衰老和异常基底细胞的存在。主要疗法是口服抗纤维化药物吡非尼酮和尼达尼布，它们可以提高生活质量，减轻症状，减缓疾病进展。单侧或双侧肺移植是 IPF 唯一能延长预期寿命的治疗方法。结论显然，对 IPF 机制的加速研究尚未得到满足，以便在治疗方面取得进展，以实现增加预期寿命、减轻症状和改善幸福感的目标。主要疗法是口服抗纤维化药物吡非尼酮和尼达尼布，它们可以提高生活质量，减轻症状，减缓疾病进展。单侧或双侧肺移植是 IPF 唯一能延长预期寿命的治疗方法。结论显然，对 IPF 机制的加速研究尚未得到满足，以便在治疗方面取得进展，以实现增加预期寿命、减轻症状和改善幸福感的目标。主要疗法是口服抗纤维化药物吡非尼酮和尼达尼布，它们可以提高生活质量，减轻症状，减缓疾病进展。单侧或双侧肺移植是 IPF 唯一能延长预期寿命的治疗方法。结论很明显，对 IPF 机制的加速研究尚未得到满足，以便在治疗方面取得进展，以实现延长预期寿命、减轻症状和改善幸福感的目标。

"点击查看英文标题和摘要"

Idiopathic pulmonary fibrosis: Current and future treatment.

OBJECTIVES Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea. Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads inexorably to respiratory failure and death within 3-5 years of diagnosis. This review discusses treatment approaches to IPF in current use and those that appear promising for future development. DATA SOURCE The data were obtained from the Randomized Controlled Trials and scientific studies published in English literature. We used search terms related to IPF, antifibrotic treatment, lung transplant, and management. RESULTS Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells. The mainstay therapies are the oral antifibrotic drugs pirfenidone and nintedanib, which can improve quality of life, attenuate symptoms, and slow disease progression. Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy. CONCLUSION Clearly, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving well-being.

更新日期：2022-01-10

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