Emerging Therapies in Pulmonary Fibrosis.

IF 3.4 Q2 RESPIRATORY SYSTEM

Pulmonary Therapy Pub Date : 2026-03-01 Epub Date: 2026-02-24 DOI:10.1007/s41030-026-00349-y

Hugh Etchingham-Coll, Ekrem Temizel, Neso Okezie-Enyioma, Nieve Martin, Punchalee Kaenmuang, Sean Coghlan, Paolo Spagnolo, Nazia Chaudhuri

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Abstract

Interstitial lung diseases (ILD) are a heterogenous group of respiratory disorders with varying degrees of inflammation and fibrosis. Idiopathic pulmonary fibrosis (IPF), the commonest and most debilitating type of ILD, is a chronic, progressive disease of the respiratory system characterized by fibrosis of the alveolar interstitium. Subsequent, relentless decline in lung function leads to progressive breathlessness and respiratory failure. Treatment options for IPF have remained mostly unchanged in the last decade, with the availability of two antifibrotic therapies: pirfenidone and nintedanib. Recently, the US Food and Drugs Administration (FDA) approved nerandomilast for the management of IPF and progressive pulmonary fibrosis. Nintedanib is also globally approved for the treatment of progressive non-IPF ILDs. While these therapies have been shown to reduce the decline of lung function, they do not reverse existing lung damage or fully address the complex pathophysiology of pulmonary fibrosis (PF). Accordingly, research in the field has shifted to developing new therapies with improved efficacy and minimal adverse effects that directly target the intricate pathogenesis in PF with the aim of arresting or reversing the disease. This review article will set the scene by first describing the pathogenesis and prevalence of ILDs, followed by exploring the current and emerging therapies in the field.

Abstract Image

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肺纤维化的新疗法。

间质性肺疾病（ILD）是一种异质性的呼吸系统疾病，具有不同程度的炎症和纤维化。特发性肺纤维化（Idiopathic pulmonary fibrosis， IPF）是一种以肺泡间质纤维化为特征的慢性进行性呼吸系统疾病，是最常见和最使人衰弱的ILD类型。随后，肺功能持续下降导致进行性呼吸困难和呼吸衰竭。在过去十年中，IPF的治疗选择基本保持不变，有两种抗纤维化治疗：吡非尼酮和尼达尼布。最近，美国食品和药物管理局（FDA）批准nerandomilast用于治疗IPF和进行性肺纤维化。Nintedanib也被全球批准用于治疗进行性非ipf fields。虽然这些疗法已被证明可以减少肺功能的下降，但它们不能逆转现有的肺损伤或完全解决肺纤维化（PF）的复杂病理生理。因此，该领域的研究已转向开发具有更高疗效和最小副作用的新疗法，直接针对PF中复杂的发病机制，目的是阻止或逆转疾病。这篇综述文章将首先描述ild的发病机制和流行情况，然后探讨该领域目前和新兴的治疗方法。

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来源期刊

Pulmonary Therapy Medicine-Pulmonary and Respiratory Medicine

CiteScore

5.20

自引率

3.30%

发文量

审稿时长

6 weeks

期刊介绍： Aims and Scope Pulmonary Therapy is an international, open access, peer-reviewed (single-blind), and rapid publication journal. The scope of the journal is broad and will consider all scientifically sound research from pre-clinical, clinical (all phases), observational, real-world, and health outcomes research around the use of pulmonary therapies, devices, and surgical techniques. Areas of focus include, but are not limited to: asthma; chronic obstructive pulmonary disease; idiopathic pulmonary fibrosis; pulmonary hypertension; cystic fibrosis; lung cancer; respiratory tract disorders; allergic rhinitis and other respiratory allergies; influenza, pneumococcal infection, respiratory syncytial virus and other respiratory infections; and inhalers and other device therapies. The journal is of interest to a broad audience of pharmaceutical and healthcare professionals and publishes original research, reviews, case reports/series, trial protocols and short communications such as commentaries and editorials. Pulmonary Therapy will consider all scientifically sound research be it positive, confirmatory or negative data. Submissions are welcomed whether they relate to an international and/or a country-specific audience, something that is crucially important when researchers are trying to target more specific patient populations. This inclusive approach allows the journal to assist in the dissemination of quality research, which may be considered of insufficient interest by other journals. Rapid Publication The journal’s publication timelines aim for a rapid peer review of 2 weeks. If an article is accepted it will be published 3–4 weeks from acceptance. The rapid timelines are achieved through the combination of a dedicated in-house editorial team, who manage article workflow, and an extensive Editorial and Advisory Board who assist with peer review. This allows the journal to support the rapid dissemination of research, whilst still providing robust peer review. Combined with the journal’s open access model this allows for the rapid, efficient communication of the latest research and reviews, fostering the advancement of pulmonary therapies. Open Access All articles published by Pulmonary Therapy are open access. Personal Service The journal’s dedicated in-house editorial team offer a personal “concierge service” meaning authors will always have an editorial contact able to update them on the status of their manuscript. The editorial team check all manuscripts to ensure that articles conform to the most recent COPE, GPP and ICMJE publishing guidelines. This supports the publication of ethically sound and transparent research. Digital Features and Plain Language Summaries Pulmonary Therapy offers a range of additional features designed to increase the visibility, readership and educational value of the journal’s content. Each article is accompanied by key summary points, giving a time-efficient overview of the content to a wide readership. Articles may be accompanied by plain language summaries to assist readers who have some knowledge of, but not in-depth expertise in, the area to understand the scientific content and overall implications of the article. The journal also provides the option to include various types of digital features including animated abstracts, video abstracts, slide decks, audio slides, instructional videos, infographics, podcasts and animations. All additional features are peer reviewed to the same high standard as the article itself. If you consider that your paper would benefit from the inclusion of a digital feature, please let us know. Our editorial team are able to create high-quality slide decks and infographics in-house, and video abstracts through our partner Research Square, and would be happy to assist in any way we can. For further information about digital features, please contact the journal editor (see ‘Contact the Journal’ for email address), and see the ‘Guidelines for digital features and plain language summaries’ document under ‘Submission guidelines’. For examples of digital features please visit our showcase page https://springerhealthcare.com/expertise/publishing-digital-features/ Publication Fees Upon acceptance of an article, authors will be required to pay the mandatory Rapid Service Fee of €4500/ $5100/ £3650. The journal will consider fee discounts and waivers for developing countries and this is decided on a case by case basis. Peer Review Process Upon submission, manuscripts are assessed by the editorial team to ensure they fit within the aims and scope of the journal and are also checked for plagiarism. All suitable submissions are then subject to a comprehensive single-blind peer review. Reviewers are selected based on their relevant expertise and publication history in the subject area. The journal has an extensive pool of editorial and advisory board members who have been selected to assist with peer review based on the afore-mentioned criteria. At least two extensive reviews are required to make the editorial decision, with the exception of some article types such as Commentaries, Editorials, and Letters which are generally reviewed by one member of the Editorial Board. Where reviewer recommendations are conflicted, the editorial board will be contacted for further advice and a presiding decision. Manuscripts are then either accepted, rejected or authors are required to make major or minor revisions (both reviewer comments and editorial comments may need to be addressed). Once a revised manuscript is re-submitted, it is assessed along with the responses to reviewer comments and if it has been adequately revised it will be accepted for publication. Accepted manuscripts are then copyedited and typeset by the production team before online publication. Appeals against decisions following peer review are considered on a case-by-case basis and should be sent to the journal editor. Preprints We encourage posting of preprints of primary research manuscripts on preprint servers, authors’ or institutional websites, and open communications between researchers whether on community preprint servers or preprint commenting platforms. Posting of preprints is not considered prior publication and will not jeopardize consideration in our journals. 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For more information about the Creative Commons Attribution-Noncommercial License, click here: http://creativecommons.org/licenses/by-nc/4.0. Contact For more information about the journal, including pre-submission enquiries, please contact christopher.vautrinot@springer.com.