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更新于：2026-04-27

Pirfenidone

吡非尼酮

更新于：2026-04-27

概要

基本信息

药物类型

小分子化药

别名

Pirfenidone (JAN/USAN/INN)、AMR-69、AP-01

+ [19]

靶点-

作用方式-

作用机制-

治疗领域

呼吸系统疾病其他疾病肿瘤+ [7]

在研适应症

特发性肺纤维化系统性硬化相关的间质性肺病尘肺病+ [21]

非在研适应症

同种异体移植排斥反应抗中性粒细胞胞质抗体相关性血管炎石棉肺+ [16]

原研机构

InterMune, Inc.

在研机构

北京康蒂尼药业股份有限公司Viatris Ltd. (New Zealand)

Avalyn Pharma, Inc.

+ [9]

非在研机构

Hoffmann-La Roche, Inc.

Roche Holding AG

Genentech, Inc.

+ [3]

权益机构

Marnac, Inc.

AFT Pharmaceuticals Ltd.

The Georgia Neurosurgical Institute

+ [5]

最高研发阶段批准上市

首次获批日期

日本 (2008-10-16),

特发性肺纤维化

最高研发阶段(中国)批准上市

特殊审评突破性疗法 (美国)、孤儿药 (美国)、孤儿药 (欧盟)、孤儿药 (日本)、孤儿药 (韩国)、快速通道 (美国)

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结构/序列

分子式C12H11NO

InChIKeyISWRGOKTTBVCFA-UHFFFAOYSA-N

CAS号53179-13-8

关联

220

项与吡非尼酮相关的临床试验

NCT07388680

/ Recruiting临床2/3期

A Randomized, Double-blind, Placebo-controlled, Multicenter Phase II/III Clinical Trial on the Efficacy and Safety of Pirfenidone Capsules in the Treatment of Radiation-induced Lung Injury With or Without Immune-related Pneumonia

Radiation-induced lung injury (RILI) is one of the most common thoracic-radiotherapy complications, with an incidence as high as 31.4 %. Multiple studies have shown that RILI can adversely affect patient prognosis by disrupting treatment schedules. Moreover, the widespread clinical use of immune-checkpoint inhibitors (ICIs) has further increased pulmonary toxicity when radiotherapy (RT) is combined with ICIs. Checkpoint-inhibitor-related pneumonitis (CIP)-i.e., immune-mediated lung injury-may necessitate permanent discontinuation of ICIs, diminish survival benefit, and, in severe cases, directly threaten life. The diagnosis of both RILI and CIP is based on an integrated assessment of subjective symptoms and imaging findings.RILI typically occurs 1-3 months after completion of radiotherapy, whereas CIP may emerge at any point during treatment. The two entities share similar clinical presentations: fever, dry cough, chest tightness, dyspnoea, and pleuritic chest pain. Computed tomography (CT) is the most sensitive imaging modality. Pulmonary-function testing is another routinely used clinical metric; vital capacity, total lung capacity, forced expiratory volume in 1 s (FEV₁), and diffusing capacity of the lung for carbon monoxide (DLCO) may all decline, with DLCO being the most sensitive parameter. In advanced cases, arterial oxygen and carbon-dioxide tensions may also deteriorate.Currently, RILI is managed empirically with systemic corticosteroids and supportive care; however, this approach yields limited improvement in diffusing capacity or ventilatory function, and its ability to prevent radiation-induced pulmonary fibrosis (RPF) remains undefined. Corticosteroids also remain the mainstay of CIP therapy. Pirfenidone, a potent cytokine inhibitor, attenuates fibroblast activity by reducing production of transforming growth factor-β1 (TGF-β1), platelet-derived growth factor (PDGF), and fibroblast growth factor (FGF), thereby suppressing fibroblast proliferation and extracellular-matrix collagen synthesis. Pre-clinical efficacy studies have demonstrated robust anti-inflammatory, anti-oxidant, and anti-fibrotic effects in the lung.Because RILI and pneumonitis arising from combined radio-immunotherapy are often indistinguishable in clinical practice, and because both share pathogenetic features with idiopathic pulmonary fibrosis (IPF), the investigators initiated this phase II/III trial to address the unmet medical need for effective therapy. Building on prior pre-clinical and clinical data, the study aims to establish the optimal dose of pirfenidone capsules for RILI with or without concomitant CIP and to confirm efficacy and safety.Phase II (dose-finding): The study consists of a screening period (Day -28 to Day -1), a 168-day treatment-observation period (Day 1-Day 168), a safety follow-up (28 ± 7 days after the last dose), and subsequent disease-progression and survival follow-up. Ninety subjects with RILI, with or without CIP, who meet all eligibility criteria will be randomly assigned 1:1:1 to low-dose pirfenidone (400 mg TID), high-dose pirfenidone (600 mg TID), or matching placebo.Phase III (confirmatory): The dose of pirfenidone capsules for phase III will be determined jointly by the sponsor and investigators based on accumulated efficacy and safety data. The trial structure mirrors phase II: screening (Day -28 to Day -1), 168-day treatment-observation (Day 1-Day 168), safety follow-up (28 ± 7 days after the last dose), and disease-progression and survival follow-up. Eligible subjects with RILI ± CIP will be randomized 1:1 to receive either pirfenidone capsules (400 mg or 600 mg TID, taken with meals) or identical placebo. After completion of the 28-day post-treatment follow-up, all phase III participants will enter an extension phase for long-term survival assessment every 3 months (± 7 days).This trial will investigate the progression-free survival (PFS) and overall survival (OS) associated with pirfenidone capsules in patients with Grade 2 and 3 radiation-induced lung injury (RILI), with or without chemotherapy-induced pneumonitis (CIP).

开始日期2026-03-26

申办/合作机构

北京康蒂尼药业股份有限公司

[+1]

NCT06241560

/ Recruiting临床2期

An Open-label, Single-group Trial to Evaluate the Effect of Pirfenidone on the Pharmacokinetics of a Single Oral Dose of BI 1015550

This study is open to adults with idiopathic pulmonary fibrosis (IPF) who are 40 years and older. The purpose of this study is to find out whether a medicine called pirfenidone changes the amount of a medicine called BI 1015550 in the blood. Some people may take more than one medicine at a time. Therefore, it is important to understand how different medicines influence one another.
Participants take one dose of BI 1015550 as a tablet. Participants then take one tablet of pirfenidone 3 times a day for one week. The dose is then increased to 2 tablets 3 times a day for the second week. In the third week the dose is increased further to 3 tablets 3 times a day. Participants then take another dose of BI 1015550 as a tablet.
Participants are in the study for a little over 1 month. During this time, they visit the study site 15 times. Two of the visits include overnight stays at the study site. The study staff also contacts the participants by phone. During the visits, the doctors collect information about participants' health and take blood samples from the participants. They compare the amount of pirfenidone and BI 1015550 in the blood. Doctors also regularly check participants' health and take note of any unwanted effects.

开始日期2026-03-05

申办/合作机构

Boehringer Ingelheim GmbH

NCT07454291

/ Not yet recruiting临床1期

A Phase 1, Open-Label Study to Evaluate Pharmacokinetics and Drug-drug Interactions of ENV-101 in Healthy Participants

The purposes of this study are to:
1. evaluate potential interactions between taladegib (ENV-101) and current standard-of-care (SOC) therapies for idiopathic pulmonary fibrosis (IPF), including nintedanib and pirfenidone, and
2. more fully characterize the pharmacokinetics (PK) of taladegib (i.e., how the body absorbs, distributes, metabolizes and excretes taladegib).
This study will enroll 4 cohorts (groups) of participants. Each cohort will experience a different duration of treatment and sequestering (being housed) at the clinical site, followed by a 14-day follow-up period for safety evaluation. The longest duration of treatment for any cohort is 30 days.

开始日期2026-03-01

申办/合作机构

Endeavor BioMedicines, Inc

100 项与吡非尼酮相关的临床结果

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100 项与吡非尼酮相关的转化医学

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100 项与吡非尼酮相关的专利（医药）

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2,282

项与吡非尼酮相关的文献（医药）

2026-12-01·MOLECULAR BIOLOGY REPORTS

Association of Pirfenidone and its Cardioprotective effects in Pentylenetetrazole-induced kindling model of Epilepsy through the High mobility group Box-1/Toll-Like Receptor-4 pathway

Article

作者: Vohora, Divya ; Majid, Haya ; Nidhi ; Dahalia, Mansi

2026-06-01·ARCHIVES OF MEDICAL RESEARCH

Pirfenidone as a Pleiotropic Antifibrotic Agent in Metabolic Steatohepatitis: From Mechanisms to Clinical Evidence

Review

作者: Méndez-Sánchez, Nahum ; Ramírez-Mejía, Mariana M ; Poo, Jorge L ; Ponciano-Rodríguez, Guadalupe

Metabolic dysfunction-associated steatohepatitis (MASH) is a progressive phenotype of steatotic liver disease that can lead to advanced fibrosis, cirrhosis and liver-related complications. Pirfenidone is a small synthetic molecule that was originally approved for idiopathic pulmonary fibrosis. It has pleiotropic antifibrotic, anti-inflammatory, antioxidant, and immunomodulatory properties. In preclinical liver models, pirfenidone reduces hepatic stellate cell activation, collagen deposition, oxidative stress and proinflammatory cytokine expression, largely through the modulation of transforming growth factor β (TGF-β), platelet-derived growth factor (PDGF), mitogen-activated protein kinases, and the Nrf2 axis. Early clinical trials, including the large prospective cohort PROMETEO, the randomized controlled trial in compensated cirrhosis ODISEA, and the translational trial in post-sustained viral response fibrosis MINERVA, show improvements in noninvasive fibrosis markers, liver function tests, quality of life, and parallel epigenetic remodeling. These data suggest that pirfenidone acts on central fibrogenic biology and may contribute to histological regression in advanced disease. Larger and longer trials, as well as rational combinations with metabolic agents, are needed to define its therapeutic role in MASH.

2026-06-01·INTERNATIONAL IMMUNOPHARMACOLOGY

Silicosis amelioration by chaetocin: a novel therapeutic strategy targeting the HIF1α-mediated PI3K/AKT/MMP9 pathway

Article

作者: Liang, Chao ; Tao, Huihui ; Fang, Yujing ; Cai, Xiaolong ; Xu, Na ; Hu, Dong ; Hu, Xiaofei ; Bai, Ying ; Jing, Yifan

Silicosis, a debilitating occupational lung fibrosis caused by chronic silica dust inhalation, currently lacks effective therapeutic interventions, and its underlying molecular pathogenesis remains incompletely understood. To address this critical gap, we employed an integrated strategy combining bioinformatics, network pharmacology, and experimental validation to systematically identify novel therapeutic targets and candidate compounds for silicosis. Through analysis of silicosis-related differentially expressed genes from the GEO database and in silico prediction using the cMap platform, we identified the natural product chaetocin as a promising candidate. In a murine model of silicosis, chaetocin administration significantly improved pulmonary function and alleviated lung inflammation and collagen deposition. Notably, at a dose of 1 mg/kg, chaetocin demonstrated superior efficacy to pirfenidone, a current standard-of-care agent. In vitro studies further confirmed that chaetocin effectively attenuated silica-triggered macrophage inflammatory responses, apoptosis, and reactive oxygen species (ROS) production, while also suppressing fibroblast migration and activation. Mechanistic investigations revealed that chaetocin exerts its anti-inflammatory and anti-fibrotic effects by directly targeting HIF1α, thereby inhibiting the downstream PI3K/AKT/MMP9 signaling axis. Collectively, our findings establish chaetocin as a novel naturally-derived therapeutic candidate with significant potential for the treatment of silicosis.

693

项与吡非尼酮相关的新闻（医药）

2026-04-27

·贝壳社

呼吸“新星”，即将纳斯达克上市

▲点击图片，了解详情作者：贝壳社近日，临床阶段生物药企Avalyn Pharma正式冲刺纳斯达克，预计上市时间为2026年4月30日，拟通过IPO筹集约2亿美元资金。本次募资将主要用于针对其特发性肺纤维化（IPF）等罕见呼吸系统疾病的吸入型改良制剂管线，全面推向关键的后期临床阶段（Phase2b/3期）。在靶点机制已被验证的前提下，通过给药途径的改变来解决真实世界的耐受性痛点，正在成为一条兼具高商业确定性与深厚工业壁垒的破局路径。 01 为何需要“吸入型”抗纤维化药物？肺纤维化被称为“不是癌症的癌症”，尤其是特发性肺纤维化（IPF），其疾病特征为肺组织进行性瘢痕化，最终导致不可逆的肺功能衰竭，其5年生存率不足30%，致死性甚至高于多种常见恶性肿瘤。在此背景下，全球抗纤维化药物市场一直具备极高的未满足临床需求。目前，临床上主要依赖已获批的口服小分子药物，如罗氏的吡非尼酮（Pirfenidone）和勃林格殷格翰的多靶点酪氨酸激酶抑制剂尼达尼布（Nintedanib）等。虽然这些口服抗纤维化药物能减缓纤维化进展，但无法完全阻止病情进展。此外，其使用常常受系统性递送带来的耐受性挑战。由于副作用，约50%开始接受现有治疗的患者在一年内停止治疗。尽管使用有限，这两款药物2024年全球总销售额超过40亿美元。以口服吡非尼酮为例，由于其需要通过高剂量口服以在肺部达到有效治疗浓度，药物不可避免地在全身血液循环中产生高暴露量。进而引发胃肠道反应（恶心、呕吐、腹泻）以及潜在的肝毒性和光过敏。这正是Avalyn切入该赛道的核心底层逻辑，不改变已被验证的有效分子结构（规避早期生物学机制失败的风险），而是通过改变给药途径，实现药代动力学（PK）分布的根本性重塑。通过气雾化吸入给药，药物可以直接靶向沉积在下呼吸道和肺部病灶，在局部形成高浓度的同时，将进入外周血的系统性暴露量降至最低，从而在不妥协疗效的前提下，大幅削减甚至消除全身性副作用，最终提升患者的用药依从性。 02 从单药改良到复方破局基于上述逻辑，Avalyn依托其在呼吸吸入领域的研发平台，构建了层次分明且具备较高商业壁垒的研发管线。此次IPO招股书显示，资金将主要集中用于以下三大核心项目的推进。作为进度最快的主力管线，AP01（吸入式吡非尼酮）在ATLAS（一项针对IPF患者的1b期临床试验）以及一项针对IPF和PPF患者的OLE试验中展现出一定的耐受性和临床活性数据。其中，ATLAS是一项随机、开放标签试验，用于比较AP01 50毫克每日一次（QD）和AP01 100毫克每日两次（BID）数据。其中，AP01两种剂量方案的副作用率都很低，包括胃肠毒性和肝酶升高。在48周时，高剂量AP01实现了肺功能近乎稳定的水平（以强制肺活量（FVC）衡量）。此外，高分辨率计算机断层扫描（HRCT）成像分析显示，超过70%的受评估患者纤维化稳定或改善，肺部瘢痕减少，肺容量改善。长期数据显示，ATLAS的耐受性特征和临床活性数据一致。OLE试验已进行超过四年半，最初100名患者中有23人继续接受AP01 100 mg BID治疗。许多持续患者诊断后超过六年，超过肺纤维化患者诊断后三至五年的中位生存期，其他患者因死亡率、疾病进展或其他临床因素而停药，包括一名因耐受性问题停药的患者。目前，Avalyn正在启动MIST，这是一项全球性的随机2b期临床试验，拟用于评估AP01在PPF中的应用，初步数据预计将在2027年下半年公布。Avalyn计划将约1.5亿美元用于推进AP01的研发。 AP02（吸入式尼达尼布）作为另一种重磅抗纤维化药物，其机制是通过抑制VEGFR、FGF和PDGF等激酶来阻断纤维化进程。然而其口服剂型的腹泻发生率极高。AP02的开发策略与AP01同源，目前正在进行名为AURA的Phase2期针对IPF患者的临床评估。 Avalyn计划将约9000万美元（结合账面现有资金及IPO募资）用于AP02的后续临床开发，进一步覆盖当前主流的抗纤维化机制。 AP03（吸入型“吡非尼酮+尼达尼布”固定剂量复方制剂），这是Avalyn管线中最具想象力、同时也最体现“吸入制剂”独特价值的一步。在临床实践中，由于口服吡非尼酮和口服尼达尼布的毒副作用会产生叠加效应。然而，由于AP01和AP02将两者的系统性毒性降至极低，双药吸入联用在安全性上具备了现实可行性。Avalyn计划投资约1000万美元，在2026年将AP03推进至Phase1期临床。 03 竞争格局在当前的IPF研发赛道中，存在着两条截然不同的竞争路线。一条是探索新靶点（全新机制，如Pliant Therapeutics的整合素抑制剂bexotegrast，或勃林格殷格翰正在研发的PDE4B抑制剂）。这类First-in-class路径虽然天花板极高，但面临着巨大的临床疗效转化风险。另一条就是Avalyn所代表的路线：锁定确定性极高的验证靶点，死磕给药途径改良。从全球BD的视角来看，呼吸与免疫领域一直是MNC，如阿斯利康、GSK、赛诺菲等的核心阵地。这些巨头在呼吸科拥有极为庞大的商业化网络和既有医生关系。一旦Avalyn在2027年的Phase 2b期数据能够确证显著的耐受性优势和稳定的肺功能保护效果，它将成为极具吸引力的并购或重磅独家授权标的。MNC可以利用自身的成熟供应链解决商业化量产，并迅速将产品铺入其呼吸科产品线的销售矩阵中。梳理Avalyn的融资历程，从2020年4月的3550万美元B轮，到2023年9月的1.75亿美元C轮，再到2025年7月由Suvretta Capital Management和SR One联合领投的1亿美元D轮。截至2026年初，其账面有约1.38亿美元现金储备。在经历了创新药估值的周期性回调后，其投资逻辑正在向能切实解决临床痛点、且商业转化路径清晰的务实资产倾斜。 Avalyn Pharma正在进行对特发性肺纤维化治疗手段的一次突围尝试，在某些特定疾病领域，“新机制”或许并非唯一出路，基于成熟靶点结合高难度制剂工程（如吸入、透皮、长效微球等），同样能孕育出具有全球竞争力的重磅资产。随着后续临床数据的逐步披露，这场通过“改变给药方式来改变疾病结局”的产业试验，其结果值得关注。往期推荐 1 贝壳观察：行业观察 2 贝壳时刻 _ _ _ *声明：本文仅用于分享，不构成任何投资建议，且非治疗方案推荐。素材来源官方媒体/网络新闻关于贝壳社贝壳社（Bioclub）是国内领先的医健创新创业服务平台。构建了"产业空间+创业培育+投资孵化+资本赋能"四位一体服务体系，为医健领域创业企业提供全周期赋能。通过深度挖掘高成长项目、精准匹配产业资源及全球化生态网络，覆盖企业从孵化培育到加速发展的全链路，重点提供包括空间落地、创业培育、资本运作及跨境资源链接服务。贝壳社以加速科学技术成果转化与产业升级为目标，致力于成为医健领域的孵化器、加速器、连接器。

IPO申请上市临床1期临床结果

2026-04-27

·BioSpace

Avalyn Strengthens Leadership Team to Support Long-Term Growth with Appointments of Industry Leaders Adam Golden and Frank Salisbury

BOSTON, April 27, 2026 (GLOBE NEWSWIRE) -- Avalyn Pharma Inc., a clinical-stage biopharmaceutical company pioneering inhaled therapies to transform the treatment paradigm for serious, rare respiratory diseases, today announced two appointments that further strengthen its leadership team as the company advances its clinical programs and prepares for future commercialization. Adam Golden, a seasoned legal and business adviser with over three decades of experience advising biopharma companies, was appointed General Counsel and Head of Business Development. Frank Salisbury, an established leader in launching multiple FDA-approved therapies, including ESBRIET ® (pirfenidone) for idiopathic pulmonary fibrosis (IPF), was appointed Senior Vice President, Commercial. “We are thrilled to welcome Adam and Frank to Avalyn at this important stage in our evolution,” said Lyn Baranowski, Chief Executive Officer of Avalyn. “Adam brings deep legal experience guiding life sciences companies through critical inflection points involving business development and legal strategy, all of which will be invaluable as we transition to life as a public company. Frank has a proven track record of building and leading commercial organizations for innovative respiratory treatments, including oral pirfenidone for IPF and sotatercept for PAH. Their expertise strengthens our ability to execute across corporate strategy, business operations, and commercial readiness as we advance our pipeline and position Avalyn for long-term success.” Adam Golden has over 30 years of experience advising biopharma companies, spanning legal, business development, and corporate strategy. Prior to joining Avalyn, Mr. Golden was a Partner and Global Head of Life Sciences at Freshfields LLP, where he founded and led the build-out of an internationally recognized life sciences practice. Previously, he served as Partner and Head of the New York Corporate Practice Group at Hogan Lovells, and spent over two decades at Kaye Scholer, where he held multiple roles, including Partner and Co-Chair, Corporate Department. Over the course of his career, Mr. Golden has advised on mergers and acquisitions, strategic collaborations, corporate governance, and SEC compliance. He currently serves on the Board of Advisors for Life Science Cares. Mr. Golden holds a J.D. from New York University School of Law and an A.B. in Chemistry from Princeton University. Frank Salisbury brings more than 24 years of commercial leadership experience across biotechnology and large pharmaceutical companies, with a focus on rare respiratory diseases. Over the course of his career, he has played a key role in the launch and commercialization of multiple therapies. At InterMune/Genentech, he led the U.S. launch and served as National Head of Sales for ESBRIET ® (pirfenidone) in IPF. He also held senior commercial roles at Acceleron Pharma, where he supported the launch of WINREVAIR ® for pulmonary arterial hypertension (PAH), as well as at Actelion, where he played a core role in the commercialization of OPSUMIT ® (macitentan) for PAH. He joins Avalyn from PureTech Health, where he was the Senior Vice President for Commercial and Product Strategy. Mr. Salisbury earned a B.A. in International Relations and an M.B.A. from Stanford University, and was a Fulbright Scholar in Germany, where he studied HIV prevention interventions. About Avalyn Pharma Avalyn aims to transform the treatment paradigm for pulmonary fibrosis and other serious, rare respiratory diseases. The company is advancing optimized inhaled formulations of established antifibrotic medicines designed to deliver drug directly to the lungs, enhance local efficacy, and reduce systemic side effects. Avalyn’s AP01 program is an optimized inhaled formulation of pirfenidone currently being evaluated in MIST, a global Phase 2b clinical trial in patients with progressive pulmonary fibrosis (PPF). AP01 has demonstrated encouraging tolerability and clinical activity across Phase 1b and an ongoing, multi-year open-label extension trial, with long-term data supporting the potential to preserve lung function while improving tolerability relative to historical oral pirfenidone data. Avalyn’s AP02 program is an optimized inhaled formulation of nintedanib currently being evaluated in AURA, a global Phase 2 clinical trial in patients with idiopathic pulmonary fibrosis (IPF). Avalyn is also advancing AP03, an inhaled fixed-dose combination of pirfenidone and nintedanib, designed to deliver dual antifibrotic mechanisms through a single lung-targeted platform. By leveraging its proprietary drug-device approach and deep expertise in rare respiratory disease development, Avalyn aims to establish a new standard of care in pulmonary fibrosis through inhaled, lung-targeted therapies. For more information, please visit avalynpharma.com and follow the company on LinkedIn. Investor Contact: Cassie Saitow, Avalyn Pharma Inc. Sr. Director, IR and Corporate Communications ir@avalynpharma.com Media Contact: Kat Lippincott, Deerfield Group kat.lippincott@deerfieldgroup.com media@avalynpharma.com

临床2期高管变更

2026-04-26

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速览！2026 CSCO 小细胞肺癌指南更新要点

引言2026 年全国 CSCO 指南会于 4 月 24-25 日在哈尔滨隆重召开。本次会议正式推出了新版诊疗指南，邀请全国肿瘤领域权威专家，通过专题授课、学术报告等多种形式，对新版指南的内容展开系统、全面的深度解析。本届会议上，《中国临床肿瘤学会（CSCO）小细胞肺癌（SCLC）诊疗指南 2026 版》重磅发布。上海市老年医学中心（复旦大学附属中山医院闵行院区）胡洁教授、中国人民解放军总医院汪进良教授、中国医学科学院肿瘤医院毕楠教授，分别围绕《2026 版 CSCO SCLC 诊疗指南》中病理及分子病理、内科、放疗部分的更新要点进行了详细解读。「丁香园肿瘤时间」特将 2026 版 CSCO SCLC 诊疗指南更新要点整理如下，旨在与各位读者一同交流学习！PART 01 SCLC 病理及分子病理更新要点1 病理学诊断1）病理学诊断新增：SCLC 免疫组织化学诊断 III 级推荐：神经内分泌分化标记物 INSM1、SCLC 常见表达异常蛋白 P53 和 RB1表 1. 2026 版 SCLC 诊疗指南病理学诊断（表格中紫色部分为更新内容）2）SCLC 病理诊断报告原则新增：SCLC 病理诊断报告原则中需关注是否存在其他非小细胞癌组织学类型成分（必备信息），具有非典型形态时加备注（可选信息）。表 2. 2026 版 SCLC 诊疗指南病理诊断报告原则（表格中紫色部分为更新内容）2 分子标志物新增：分子标志物检测中 SCLC 的二线治疗（6 个月以上复发）新增肿瘤标志物 DLL3（III 级推荐，2B 类）表 3. 2026 版 SCLC 诊疗指南分子标志物（表格中紫色部分为更新内容）注释中新增 DLL3 靶点、SEZ6 靶点、B7 H3 靶点、MHC-I 相关特征与免疫治疗、外周血蛋白组学新证据、多组学联合分析新证据。具体如下：表 4. 2026 版 SCLC 诊疗指南分子标志物注释部分更新PART 02SCLC 内科治疗更新要点1 局限期 SCLC 更新肿瘤分期超过 T1-2，N0 的局限期 SCLC 治疗中，「同步放化疗后无进展患者，度伐利尤单抗巩固治疗 2 年」推荐等级由「II 级推荐」上调为「Ⅰ 级推荐」。表 5. 2026 版 SCLC 诊疗指南局限期 SCLC 治疗（表格中紫色部分为更新内容）2 广泛期 SCLC 初治及一线维持治疗更新1. 无局部症状且无脑转移患者一线及维持治疗 I 级推荐中，调整化疗 + 免疫治疗（I 级推荐）作为「优选」推荐；2. 无局部症状且无脑转移患者一线及维持治疗 I 级推荐中，新增「索卡佐利单抗 + 依托泊苷 + 卡铂 4 周期后索卡佐利单抗维持治疗」为「I 级推荐」方案；3. 无局部症状且无脑转移患者一线及维持治疗 II 级推荐中，新增「阿替利珠单抗 + 依托泊苷 + 卡铂 4 周期后芦比替定联合阿替利珠单抗维持治疗」为「II 级推荐」方案；4. 新增「伴骨转移」分层，补充了骨保护药物的临床建议。表 6. 2026 版 SCLC 诊疗指南广泛期 SCLC 初治及一线维持治疗（表格中紫色部分为更新内容）表 7. 2026 版 SCLC 诊疗指南脑转移患者分层治疗推荐（表格中紫色部分为更新内容）3 广泛期 SCLC 后线治疗➤ 二线治疗1. 广泛期 SCLC 二线治疗推荐删除 Ⅲ 级推荐「苯达莫司汀」（2B 类）；2. 广泛期 SCLC 二线治疗推荐新增「塔拉妥单抗」为 Ⅱ 级推荐（1A 类）。表 8. 2026 版 SCLC 诊疗指南广泛期 SCLC 二线治疗（表格中紫色部分为更新内容）➤ 三线及以上治疗广泛期 SCLC 三线及以上治疗新增「塔拉妥单抗」为 Ⅱ 级推荐（1A 类）；表 9. 2026 版 SCLC 指南广泛期 SCLC 三线及以上治疗（表格中紫色部分为更新内容）PART 03SCLC 放疗更新要点1 局限期 SCLC 初始治疗➤ 局限期 SCLC 胸部放疗注释（4）新增了证据（新增紫色部分）：放疗总剂量和分割方案：目前尚未确定最佳的放疗剂量和分割方案。根据 INT0096 研究，45 Gy/1.5 Gy，每天 2 次 /3 周方案优于 45 Gy/1.8 Gy，每天 1 次 /5 周方案。而两项 III 期研究 CONVERT 研究和 RTOG0538 研究均未能证明 66 Gy 或 70 Gy（每天 1 次）方案优于 45 Gy（每天 2 次）方案，但前者的总生存率和毒性均与后者相似 [13, 30]，因此推荐局限期 SCLC 患者胸部放疗总剂量为 45 Gy/1.5 Gy，每天 2 次 /3 周或总剂量为 60～70 Gy，1.8～2.0 Gy，每天 1 次 /（6～8 周）。回顾性和随机 II 期研究表明，加速大分割方案总剂量为 40～42 Gy（每天 1 次，3 周完成）可产生与 45 Gy/1.5 Gy，每天 2 次相似的结果。一项中国 III 期研究显示 45 Gy/3 Gy/15 次的大分割放疗方案与高剂量常规分割放疗相比（60 Gy/2 Gy/30 次）生存相似；但大分割放疗组显示出更高的安全性，≥3 级急性治疗相关不良事件的发生率显著较低（48.7% vs. 67.7%，P<0.001），≥2 级放射性肺炎（radiation pneumonia, RP）发生率也显著降低（7.7% vs. 14.5%，P=0.001）。近期随机对照 II 期研究和回顾性研究显示，如果可以安全地给予更高剂量（60 Gy/40 次，每天 2 次]；65 Gy/26 次，每天 1 次；或肿瘤同步加量至 54 Gy/30 次，每天 2 次），可能改善 OS 或 PFS。一项中国 III 期随机对照临床研究（NCT03214003）对比了高剂量 [计划靶区（planning target volume，PTV）给予 45 Gy/30 次，大体肿瘤靶区（gross tumor target volume, GTV）同步加量至 54 Gy/30 次，每天 2 次）] 和标准剂量（PTV 均一给量 45 Gy/30 次，每天两次）两种超分割方案，初步结果显示高剂量超分割方案可显著延长 OS 或 PFS。➤ 局限期 SCLC 内科治疗注释（5）新增了证据（新增紫色部分）：ADRIATIC 研究的成功也引发了关于 LS-SCLC 研究中免疫治疗介入时机、与免疫治疗联合的最佳胸部放疗分割方式，免疫治疗的不同药物和免疫治疗的时长，PCI 在 LS-SCLC 免疫治疗中的疗效和安全性，选择卡铂还是顺铂能够与 LS-SCLC 免疫治疗发挥最大协同作用等问题的深入思考。在免疫巩固治疗方面：ASTRUM-LC01 II 期研究（斯鲁利单抗巩固治疗 1 年）和 GASTO-1052 II 期研究（特瑞普利单抗巩固治疗 6 个月）均证实在 LS-SCLC 同步放化疗后使用免疫巩固治疗显著提高疗效（前者 PFS 中位数为 27.5 个月，后者尚未达到）。而 ACHILES II 期研究在同步放化疗后使用阿替利珠单抗巩固治疗未观察到生存获益。正在进行的 DeLLphi-306、HLX10-020-SCLC302、MK 7339-013/KEYLYNK-013 等研究结果值得期待。NRG LU005 研究同步放化疗中联合阿替利珠单抗治疗与同步放化疗相比并没有看到生存获益。目前免疫治疗从诱导治疗开始就介入的研究 SHR-1316-III-302 研究、HLX10-020-SCLC302 仍然在探索中。一项 II 期研究显示，卡瑞利珠单抗联合化疗诱导后序贯 cCRT 及免疫巩固治疗，在客观缓解率、无进展生存和总生存方面均优于单纯 cCRT。2 广泛期 SCLC 的初始治疗➤广泛期 SCLC 放疗治疗更新（新增紫色部分）：一项胸部低剂量放疗（low-dose radiotherapy，LDRT）15 Gy/5 次同步一线阿替利珠单抗 + 依托泊苷 / 顺铂或卡铂，并阿替利珠单抗维持治疗的 I 期试验（MATCH试验）显示出肿瘤免疫微环境的重塑，良好的安全性和可行性，以及近期和中期疗效（ORR 为 73.3%，PFS 中位数为 6.9 个月），OS 中位数为 16.9 个月。1、3 年 PFS 率分别为 27.3% 和 20.7%，1 年和 3 年 OS 率分别为 69.9% 和 35.1%。进一步，相似设计的 LDRT（15 Gy/5 次）同步一线联合化疗与免疫治疗的 2 项前瞻性多中心I期临床试验进一步报道结果，安全性和耐受性均良好，联合度伐利尤单抗的 PFS 中位数和 OS 分别为 8.3 个月和 23.6 月（LEAD 试验）；联合斯鲁利单抗的 PFS 中位数为 7.3 个月，OS 中位数尚不成熟（SPUR 试验）。表 10. 2026 版 SCLC 诊疗指南广泛期 SCLC 放疗相关表格修订3 放疗并发症的处理➤ 放射性肺损伤注释部分修改完善3）对症支持治疗更新（新增紫色部分）：止咳、化痰、平喘治疗。另外，可考虑辅助抗纤维化治疗及中医药治疗，保持充足能量供给并补充多种维生素。Ⅱ 期研究发现吡非尼酮联合标准疗法能够改善放射性肺损伤（radiation induced lung injury，RILI）患者的肺功能、轻到中度放射性肺纤维化，但目前尚无 Ⅲ 期随机对照试验证据支持常规应用，仅推荐在临床试验背景下使用。4）放射性和免疫性肺损伤共存更新（新增紫色部分）：对于（序贯或同期）合并使用免疫检查点抑制剂的患者，特别是免疫相关细胞因子明显变化的患者，在诊断放射性肺损伤的情况下，需注意同时合并免疫相关肺损伤（check point inhibitor pneumonitis, CIP）的可能性。这种复合性肺损伤的个案或回顾性报道自 2018 年以来逐步增多，部分患者糖皮质激素减量时症状反复，可能需使用其他免疫抑制剂。鉴别放射 - 免疫相关性肺炎对于其治疗至关重要。（1）在发病时机上，RILI 多在放疗后 6 个月内出现，尤其在放疗后 1～3 个月内较为常见，而 CIP 可能在治疗后 2～24 个月内的任何时间发生。（2）影像学上，RL 通常局限于放射区域内，表现为渗出实变和纤维化；CIP 的影像学表现则更为多样，可分为 5 种类型：隐源性组织性肺炎、磨砂玻璃样结节的磨玻璃样混浊、小叶间隔增厚的间质性肺炎、小叶中心结节的超敏反应性肺炎，以及未明确定义的肺炎。临床上，RILI 和 CIP 的鉴别常依据病变位置，RLI 多见于照射的高剂量区域，与放疗剂量参数紧密相关，而 CIP 则多见于高剂量区域之外。（3）在临床实践中，除了明确放射-免疫相关性肺炎的性质，还需与其他可能引起相似症状的疾病进行鉴别诊断。RIL 和 CIP 均为无菌性炎症，与感染性疾病相比，通常不伴有发热和白细胞升高等典型感染症状。感染性肺炎的影像学表现早期为毛玻璃样阴影，细菌性肺炎多局限于肺叶或肺段，而病不良反应肺炎则表现为多发毛玻璃样阴影。（4）此外，还需注意与肿瘤进展的鉴别后者常表现为咳嗽、咯血、胸痛、体重减轻、呼吸困难，肿瘤标志物可能升高，影像学上可见肺癌原发灶增大及新的结节状影、片状影、毛玻璃影等。➤ 放射性皮肤损伤注释部分修改完善更新（新增紫色部分）：因此，在进行局部治疗的同时，应积极进行全身治疗。目前在国内外相关指南和共识中，关于生长因子类或生物制剂类药物在放射性皮肤损伤预防与治疗中的应用推荐意见尚不统一。国内的相关指南、共识中，对于 EGF、GM-CSF 在放射性皮肤损伤 / 放射性皮炎中的预防或治疗性应用予以弱推荐。整理：丁香园；编辑：gulu题图：图虫创意

100 项与吡非尼酮相关的药物交易

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外链

KEGG	Wiki	ATC	Drug Bank
D01583	吡非尼酮	L04AX05	DB04951

研发状态

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适应症	国家/地区	公司	日期
特发性肺纤维化	日本	Shionogi & Co., Ltd.	2008-10-16

未上市

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适应症	最高研发状态	国家/地区	公司	日期
肺炎	临床3期	中国	北京康蒂尼药业股份有限公司	2026-03-26
辐射损伤	临床3期	中国	北京康蒂尼药业股份有限公司	2026-03-26
系统性硬化相关的间质性肺病	临床3期	中国	GNI Group Ltd.	2023-05-29
尘肺病	临床3期	中国	GNI Group Ltd.	2023-05-29
煤肺病	临床3期	中国	北京康蒂尼药业股份有限公司	2022-06-07
矽肺	临床3期	中国	北京康蒂尼药业股份有限公司	2022-05-09
硬化肼胝症	临床3期	中国	北京康蒂尼药业股份有限公司	2018-06-08
皮肌炎	临床3期	中国	北京康蒂尼药业股份有限公司	2018-06-01
间质性肺疾病	临床3期	中国	北京康蒂尼药业股份有限公司	2018-06-01
肺损伤	临床2期	中国	北京康蒂尼药业股份有限公司	2026-03-26

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研究	分期	人群特征	评价人数	分组	结果	评价	发布日期


NCT06484153 (ASCO_GI2026)	临床1/2期	转移性结直肠癌 pMMR \| MSS	24	Fruquintinib+pirfenidone+anti-PD-1 antibody	醖網壓範構餘鑰窪獵鹽(獵糧製壓願築遞築膚蓋) = 膚鬱顧窪襯鏇鏇衊窪蓋壓艱艱夢築製網築顧蓋 (夢廠鹽積廠簾鬱夢蓋廠 ) 更多	积极	2026-01-08
NCT06329401 (MIST, Pubmed \| BMJ Open Respir Res)	临床2期	进展性肺纤维化	300	AP01 50 mg two times per day	蓋簾憲衊蓋顧膚壓蓋淵(憲壓繭膚鹹選襯鹽製餘) = 餘襯範窪憲憲鑰選構衊範築壓夢廠窪醖膚醖顧 (壓廠夢夢繭遞鏇糧醖餘 )	积极	2025-12-01
				Placebo two times per day	膚艱積選顧鏇鑰艱製選(網襯觸觸艱窪醖觸廠鹹) = 夢願製築鹹廠淵衊窪蓋遞網範遞積衊鹹襯衊網 (膚糧範選艱鹹壓夢襯鬱 )
NCT03902509 (Pubmed \| Lancet Oncol)	临床2期	-	134	Pirfenidone plus glucocorticoids	築窪蓋網鹹鬱鏇糧醖選(願築鬱選鹽顧選鑰鑰衊) = pneumonia (four [6%] of 67 patients in the pirfenidone group vs eight [12%] of 67 patients in the control group), and rash (two [3%] of 67 patients in the pirfenidone group vs none in the control group). 鹹醖蓋鏇蓋鏇鏇窪醖餘 (膚範壓顧願顧鬱積齋鬱 )	积极	2025-12-01
				Glucocorticoids
ACR2025	N/A	原发性干燥综合征 anti-SSA/Ro \| anti-SSB/La	45	Glucocorticoids	憲憲糧簾夢壓顧鹹範選(廠鬱築醖壓淵廠鏇醖壓) = 選夢簾衊鏇選醖構簾憲遞蓋簾壓範觸範窪壓淵 (夢鹹製夢構膚鏇壓衊鏇, 114.0) 更多	积极	2025-10-24
ACR2025	N/A	自身免疫性疾病 \| 系统性硬皮病 \| 类风湿性关节炎伴有类风湿性肺病	132	nintedanib (RA-ILD + SSc-ILD + SADs-ILD)	繭膚憲選鹽壓襯憲願獵(構醖網築簾遞襯鹹齋網) = 衊遞構築觸糧顧糧簾窪艱觸夢膚糧膚夢鏇遞襯 (製壓餘鹽鹹積積醖築醖 ) 更多	积极	2025-10-24
				pirfenidone (RA-ILD + SSc-ILD + SADs-ILD)	繭膚憲選鹽壓襯憲願獵(構醖網築簾遞襯鹹齋網) = 構鏇願築鑰餘選積築艱艱觸夢膚糧膚夢鏇遞襯 (製壓餘鹽鹹積積醖築醖 ) 更多
ChiCTR2400081900 (ESMO2025)	临床2期	放射性口腔黏膜炎	87	Pirfenidone	觸蓋襯襯鬱製製繭顧網(蓋積憲築鹽獵廠構憲願) = 夢製壓淵製憲壓糧衊鏇壓遞襯積鑰獵選襯壓膚 (餘鹽憲蓋糧鏇廠醖簾簾 )	积极	2025-10-17
				Pirfenidone (Historical controls)	觸蓋襯襯鬱製製繭顧網(蓋積憲築鹽獵廠構憲願) = 鑰窪壓窪窪鏇製簾構獵壓遞襯積鑰獵選襯壓膚 (餘鹽憲蓋糧鏇廠醖簾簾 )
ASCO2025	N/A	特发性肺纤维化	4,529	Pirfenidone	壓窪餘襯顧積淵壓糧齋(廠壓繭餘艱壓獵衊窪窪) = 蓋築繭餘製顧簾遞範觸簾遞襯淵夢鬱鏇壓淵網 (衊糧選網憲築艱構選窪 )	积极	2025-05-30
				Non-Pirfenidone	壓窪餘襯顧積淵壓糧齋(廠壓繭餘艱壓獵衊窪窪) = 積範糧願鬱餘餘築膚夢簾遞襯淵夢鬱鏇壓淵網 (衊糧選網憲築艱構選窪 )
ATLAS Phase 1b Trial (ATS2025)	临床1期	特发性肺纤维化	64	Nebulised AP01 50mg QD	醖繭簾遞獵簾構襯選構(鹽築鹹衊繭壓膚糧餘簾) = 顧遞範糧簾網選願鑰觸鹹製獵窪製積壓築觸淵 (憲遞夢網襯齋鬱鹹範範 ) 更多	积极	2025-05-16
				Nebulised AP01 100mg BID	醖繭簾遞獵簾構襯選構(鹽築鹹衊繭壓膚糧餘簾) = 餘構鏇網構窪蓋觸鑰壓鹹製獵窪製積壓築觸淵 (憲遞夢網襯齋鬱鹹範範 ) 更多
ATLAS Phase 1b Trial (ATS2025)	临床1期	-	28	AP01 50mg QD	淵鹽選衊積憲夢廠鏇壓(壓膚顧製衊選糧網築範) = 範顧壓膚鏇築憲壓鏇廠選選醖鬱觸齋鏇鹽範壓 (選齋鏇鹽築廠選遞窪鬱, 5.61)	积极	2025-05-16
				AP01 100mg BID	淵鹽選衊積憲夢廠鏇壓(壓膚顧製衊選糧網築範) = 構齋繭築糧網夢築獵遞選選醖鬱觸齋鏇鹽範壓 (選齋鏇鹽築廠選遞窪鬱, 7.87)
AP01-002 (ATLAS) (ATS2025)	N/A	-	91	50 mg AP01 once daily	淵艱製選遞簾艱齋壓襯(積獵築蓋艱衊鬱遞艱夢) = 鑰觸醖鏇壓積壓鬱廠鑰顧襯觸網鏇鏇蓋糧壓獵 (簾獵廠壓憲範齋窪壓鹹 )	积极	2025-05-16
				100 mg AP01 twice daily	淵艱製選遞簾艱齋壓襯(積獵築蓋艱衊鬱遞艱夢) = 鹽餘繭齋獵選夢壓顧蓋顧襯觸網鏇鏇蓋糧壓獵 (簾獵廠壓憲範齋窪壓鹹 ) 更多