预约演示

更新于：2026-01-28

Nintedanib esylate

乙磺酸尼达尼布

更新于：2026-01-28

概要

基本信息

药物类型

小分子化药

别名

CYNEDIV、Intedanib、NDNB

+ [26]

靶点

CSF-1R x FGFRs x FLT3

作用方式

拮抗剂、抑制剂

作用机制

CSF-1R拮抗剂(集落刺激因子1受体拮抗剂)、FGFRs拮抗剂(成纤维细胞生长因子受体家族拮抗剂)、FLT3抑制剂(酪氨酸蛋白激酶受体FLT3抑制剂)

治疗领域

肿瘤呼吸系统疾病其他疾病+ [6]

在研适应症

局部晚期非小细胞肺癌转移性非小细胞肺癌复发性非小细胞肺癌+ [23]

非在研适应症

急性红细胞白血病急性早幼粒细胞白血病大肠腺癌+ [52]

原研机构

Boehringer Ingelheim International GmbH

在研机构

Cloudbreak Therapeutics LLC

MannKind Corp.

Boehringer Ingelheim GmbH

+ [15]

非在研机构

美莱普（北京）医药研究有限公司

权益机构

远大医药（中国）有限公司

Santen Pharmaceutical Co., Ltd.

Mount Sinai Medical Center of Florida, Inc.

+ [3]

最高研发阶段批准上市

首次获批日期

美国 (2014-10-15),

特发性肺纤维化

最高研发阶段(中国)批准上市

特殊审评快速通道 (美国)、孤儿药 (美国)、孤儿药 (日本)、孤儿药 (韩国)、孤儿药 (澳大利亚)、特殊审批 (中国)、优先审评 (中国)、突破性疗法 (美国)

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结构/序列

分子式C33H39N5O7S

InChIKeyMMMVNAGRWOJNMW-FJBFXRHMSA-N

CAS号656247-18-6

关联

245

项与乙磺酸尼达尼布相关的临床试验

NCT07335562

/ Not yet recruiting临床3期

A Phase 3, Randomized, Open-label, Multicenter Study to Compare the Efficacy and Safety of BMS-986353, CD19-targeted NEX-T CAR T Cells, Versus Standard of Care in Participants With Active Systemic Sclerosis (Breakfree-SSc)

The purpose of this study is to compare the efficacy and safety of BMS-986353 versus standard of care in participants with active Systemic Sclerosis

开始日期2026-04-15

申办/合作机构

Juno Therapeutics, Inc.

[+1]

NCT07194382

/ Not yet recruiting临床2期

A Randomized, Double-Blind, Placebo-Controlled, Phase 2 Proof of Concept (POC) Study Evaluating the Safety, Tolerability, and Efficacy of Nintedanib Solution for Inhalation (AP02) in Participants With Idiopathic Pulmonary Fibrosis (IPF) (AURA-IPF)

This study will evaluate the impact Nintedanib Solution for Inhalation (AP02) has on lung function and key measures of fibrosis in adult patients with idiopathic pulmonary fibrosis (IPF) as well as assess its safety and tolerability.
Adults 40 years of age or older with IPF who meet the inclusion and exclusion criteria can participate in this study if they are not currently on treatment for IPF, and if treated with oral nintedanib or pirfenidone, have stopped the medication for at least 3 months.
Researchers will compare two different doses of AP02 to a placebo (a look-alike substance that contains no drug) to see if AP02 works to treat IPF. Participants are put into 1 of 3 groups randomly, which means by chance and will take AP02 or a placebo two times every day for 12 weeks by using a nebulizer, which is a device that provides medicine to the lungs via inhalation.
Participants will visit the office 6 times and receive 1 phone call over a 16-week period. At site visits doctors regularly perform breathing tests that measure how well the lungs are working, give the patient questionnaires and will check the participants' health.

开始日期2026-01-01

申办/合作机构

Avalyn Pharma Inc.

NCT07344558

/ Recruiting临床1期

A Randomized, Double-Blind, Placebo-Controlled, Phase 1b Clinical Study of the Safety, Tolerability, and Pharmacokinetics of MNKD-201 (Nintedanib Dry Powder Inhalation) in Patients With Idiopathic Pulmonary Fibrosis

MKC-NI-002 is a Phase 1b, randomized, double-blind, placebo-controlled study of nintedanib inhalation powder (MNKD-201) in patients with Idiopathic Pulmonary Fibrosis (IPF). The trial consists of Multiple Ascending Doses (MAD) with the primary objective to evaluate safety, tolerability and pharmacokinetics (PK) of MNKD-201 compared to placebo in patients with IPF.

开始日期2025-12-22

申办/合作机构

MannKind Corp.

100 项与乙磺酸尼达尼布相关的临床结果

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100 项与乙磺酸尼达尼布相关的转化医学

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100 项与乙磺酸尼达尼布相关的专利（医药）

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1,942

项与乙磺酸尼达尼布相关的文献（医药）

2026-05-01·CELLULAR SIGNALLING

Anti-fibrotic activity of nobiletin and nintedanib: In vitro and in vivo evidence in pulmonary fibrosis models

Article

作者: Wang, Yuanru ; Li, Luyao ; Yang, Xue ; Li, Xuejun ; Li, Huifang ; Zhang, Xiaoqian ; Cao, Yajun ; Lei, Qiqi ; Liu, Jie ; Xiang, Liuyan

BACKGROUND:

Pulmonary fibrosis, a well-known chronic and progressive lung disease, primarily impacts the interstitial tissues of the lungs, with a lack of effective therapies. Nobiletin is a polymethoxyflavonoid that exhibits characteristics of BH3 mimetics. It is mainly extracted from citrus peels and is known for its diverse pharmacological activities. Given the unsatisfactory clinical trial results of nintedanib, a combined treatment approach may represent a viable strategy for combating pulmonary fibrosis.

METHODS:

An in vitro pulmonary fibrosis model was established by inducing MRC-5 cells with transforming growth factor-β1 (TGF-β1). The impact of nobiletin combined with nintedanib on the migration of MRC-5 cells was assessed by wound healing and Transwell assays. Immunofluorescence and Western blot analyses were employed to assess the effect of drug combination on fibrosis-related markers, while Co-Immunoprecipitation (Co-IP) experiments were performed to assess the effects on autophagy. The anti-fibrotic effects and potential mechanisms of the combination of nobiletin and nintedanib were further explored using a bleomycin-induced pulmonary fibrosis model in C57BL/6 J mice.

RESULTS:

In vitro, the combination of nobiletin and nintedanib significantly inhibited MRC-5 cells' migration and extracellular matrix deposition, while simultaneously promoting apoptosis and autophagy. In addition, this combination exerted an anti-pulmonary fibrosis effect by regulating epigenetic mechanisms and the PI3K-AKT-mTOR signaling pathway. In vivo studies further revealed that the combination of nobiletin and nintedanib significantly reduced hydroxyproline levels in mice, attenuated lung inflammation, and helped to limit or prevent collagen accumulation.

CONCLUSIONS:

Our study demonstrates that the combination of nobiletin and nintedanib exhibits promising anti-fibrotic effects both in vitro and in vivo.

2026-03-01·LUNG CANCER

Nintedanib as switch maintenance treatment in malignant pleural mesothelioma (NEMO): A double-blind randomized phase II trial (EORTC-08112-LCG)

Article

作者: Raskin, Jo ; Marreaud, Sandrine ; Hassani, Adam ; Abdel-Rahman, Omar ; Surmont, Veerle ; Taylor, Paul ; Jankowska, Petra ; Boone, Luc ; Dazzi, Claudio ; Zonato, Sabrina ; Young, Robin ; Toffart, Anne-Claire ; Popat, Sanjay ; O'Brien, Mary

INTRODUCTION:

Pleural mesothelioma (PM) is a lethal malignancy in which angiogenesis and progressive fibrosis drives disease. We evaluated angiogenesis inhibition using nintedanib monotherapy as switch maintenance in patients with PM that completed 4-6 cycles of prior platinum-pemetrexed chemotherapy. The trial was performed in parallel to the LUME-Meso trial in an era prior to routine immune checkpoint inhibitor use, when platinum-pemetrexed was standard of care.

METHODS:

This is a triple-blind, placebo-controlled, multicentric, randomized, phase II study. Patients with inoperable PM (all histologies) that completed 4-6 cycles of platinum-pemetrexed chemotherapy were randomized to nintedanib or placebo. The primary endpoint was progression-free survival (PFS). Secondary endpoints included overall survival (OS) and safety.

RESULTS:

The trial was prematurely closed due to poor accrual after LUME-meso reported. 37 patients were randomized (18 to nintedanib, 19 to placebo). All analyses performed were descriptive. The median PFS was 3.4 months (95% CI: 2.83-5.82) and 4.6 months (95% CI: 3.65-13.17) for nintedanib and placebo arms, respectively (HR = 2.25, 95% CI: 1.07-4.73) with corresponding median OS of 13.1 months (95% CI: 10.22-26.02) and 38.9 months (95% CI: 18.66-NE) for nintedanib and placebo arms, respectively (HR = 2.38, 95% CI: 1.05-5.43). Two patients (11.1%) in the nintedanib arm experienced ≥ grade 3 treatment-related adverse events. Post progression treatment was balanced between arms but more patients in the placebo arm received immunotherapy (87% vs 54%).

CONCLUSIONS:

Descriptive analyses suggest switch maintenance nintedanib does not improve PFS nor OS compared to placebo. Efficacy of the placebo arm may be related to imbalanced immunotherapy usage.

2026-03-01·JOURNAL OF CONTROLLED RELEASE

Inhalable stealth liposomes improve peptide delivery for pulmonary fibrosis treatment

Article

作者: Zhu, Yugen ; Wang, Chaoqun ; Cao, Xiang ; Li, Zhengqing ; Shen, Jianhao ; Sun, Yuelan ; Tan, Zheng ; Zhang, Jiabin ; Lin, Ruibo ; Huang, Yongzhuo ; Fu, Chang ; Shen, Shiyang ; Lin, Yongfang

Pulmonary fibrosis is a life-threatening lung disease with limited therapeutic options and suboptimal clinical outcomes. Peptide-based agents such as CSP7, a heptapeptide derived from caveolin-1, have shown promising antifibrotic activity with low systemic toxicity. However, effective inhaled administration of CSP7 is impeded by physiological barriers in fibrotic lungs, including alveolar edema fluid and clearance by inflammatory macrophages. Here, we report a high-loading stealth liposomal system designed to enhance pulmonary delivery of CSP7 by inhalation. CSP7 was covalently conjugated to an aliphatic lipid moiety to generate a lipidated prodrug (l-CSP7), enabling markedly improved incorporation into PEGylated stealth liposomes (l-CSP7/Lips) with about 4-fold higher encapsulation efficiency and drug loading. The optimized formulation also exhibited enhanced penetration through edematous alveolar fluid and reduced macrophage clearance. After deposition in the alveoli and uptake by myofibroblasts, intracellular enzymes cleave l-CSP7 to release active CSP7, thereby attenuating profibrotic cellular phenotypes. In bleomycin-induced idiopathic pulmonary fibrosis and silica-induced silicosis animal models, inhaled l-CSP7/Lips achieved superior alveolar delivery and therapeutic outcomes compared with free CSP7 and clinical drug nintedanib, reversing fibrotic remodeling, improving respiratory function, and reducing inflammation and collagen deposition. These results introduce a modular and translatable nanocarrier platform for inhalable peptide therapeutics and support a localized treatment strategy for pulmonary fibrosis and potentially other lung diseases.

692

项与乙磺酸尼达尼布相关的新闻（医药）

2026-01-27

·GlobeNewswire-Health Care

Cloudbreak Pharma Announces Successful End-of-Phase-2 Meeting and Alignment with FDA on Phase 3 Path Forward for CBT-004

CBT-004, if approved, would be the first and only treatment for pinguecula, an ophthalmic condition impacting more than 50 million people in the U.S. alone In prior studies, CBT-004 demonstrated an ability to modify disease progression through a novel, multi-target mechanism of action Phase 3 data from lead asset, CBT-001, as a potential treatment for pterygium anticipated in Q4 2026 IRVINE, Calif., Jan. 27, 2026 (GLOBE NEWSWIRE) -- Cloudbreak Pharma Inc., (HKEX 2592), a clinical stage ophthalmic drug company leveraging its proprietary Multi Kinase Inhibitor (MKI) discovery platform to develop first-in-class and best-in-class therapeutics, today announced the results of a successful End-of-Phase 2 (EOP2) meeting with the U.S. Food and Drug Administration (FDA) regarding the late-stage development of CBT-004 for the treatment of pinguecula. Francis Mah, MD, Director of Cornea and External Disease at Scripps Clinic Medical Group in La Jolla, CA, stated, “Pinguecula is a widely prevalent ocular disease characterized by a growth near the cornea that is prone to inflammation and abnormal vascularization, causing a ‘foreign body’ sensation in the eye that can interfere significantly with patient quality of life. The current standard-of-care, corticosteroids, addresses only symptoms and not the underlying disease, and presents safety challenges with longer-term use. In a prior rigorously designed Phase 2 study, CBT-004 addressed the most bothersome symptoms of pinguecula while also demonstrating an ability to modify the trajectory of the disease. I look forward to one day incorporating it into my own practice, if approved.” Abu Abraham, MD, Chief Medical Officer at Cloudbreak, added, “We are extremely pleased to have completed a successful end-of-phase 2 meeting with FDA and reached alignment with the Agency on a reasonable and clear path forward for CBT-004 that includes endpoints that were previously achieved in our successful Phase 2 study. I would once again like to thank the patients and investigators who participated in our Phase 2 program, and I am optimistic that we will be able to replicate those compelling results in upcoming pivotal studies. We are well positioned to help the millions of people across the U.S. who suffer from this bothersome condition while at the same time creating significant value for our Company in this multi-billion addressable market.” Phase 2 recapThe EOP2 meeting was supported by positive results from a multicenter, randomized, double-masked, vehicle-controlled Phase 2 study that enrolled 88 adult patients with vascularized pinguecula and associated conjunctival hyperemia. Participants were randomized to receive one of two concentrations of CBT-004, or vehicle. Both investigated concentrations of CBT-004 demonstrated statistically significant improvements in conjunctival hyperemia compared to vehicle at Day 28, the primary endpoint, as assessed by an independent reading center using digital imaging. Significant improvements were observed with the higher dose of CBT-004 as early as the first scheduled study visit on Day 7. Benefits persisted through the 28-day treatment period for both CBT-004 concentrations, showing statistically significant improvements in five common patient-reported symptoms, including burning/stinging, itching, foreign body sensation, eye discomfort, and pain compared to vehicle. No treatment-related adverse events were observed. Most adverse events were mild to moderate. No clinically meaningful changes in visual acuity or intraocular pressure were reported. Anticipated Phase 3 study designThe primary efficacy endpoints for the Phase 3 studies will include a sign and symptom of pinguecula, both of which were statistically significant findings in the prior Phase 2 trial. The current plan is for Cloudbreak to conduct a Phase 3 program in which efficacy is assessed at three months, and safety is assessed at twelve months. The company is planning to complete remaining toxicity studies in 2026 and initiate the Phase 3 program in Q1 2027. Notably, the Company continues to engage in dialogue with the FDA to determine if a path forward may exist to conduct a single, sufficiently powered pivotal study consistent with recent statements made by the FDA Commissioner. About Pinguecula Pinguecula (pin·gwek·yoo·luh) is a round, yellowish, elevated tissue that develops on the surface of the eye. The condition is very common among people with increased UV light exposure and increasing age. Routinely diagnosed by optometrists and ophthalmologists, the condition likely impacts over 50 million people in the US and a billion people worldwide. When the tissue becomes vascularized or inflamed, it can produce a number of symptoms including ocular redness, discomfort and pain, foreign body sensation, tearing and itching. Depending on the location and size of the pinguecula, it may also make wearing contact lenses uncomfortable or impossible. There is currently no pharmacological treatment for pinguecula approved by the FDA. To treat some symptoms, such as dry eye and foreign body sensation, lubricating eye drops may be used. For pinguecula that are larger or inflamed, non-steroidal anti-inflammatory drugs or corticosteroids are used, although the former can be associated with corneal complications, while the latter is associated with glaucoma and cataract formation. With few existing reliable options for symptom relief, there is a strong need for pharmacological treatment specifically designed to address this condition. About CBT-004CBT-004 is a novel, preservative-free topical ophthalmic solution containing a potent VEGF receptor inhibitor. CBT-004 also inhibits platelet-derived growth factors which are responsible for blood vessel maintenance. By inhibiting these growth factors, the formulation is specifically designed to address the hyperemia and abnormal vascularization associated with the pinguecula lesion. The preservative-free formulation is designed to minimize potential ocular surface toxicity, positioning it for possible chronic use in this condition affecting the delicate ocular surface. About CloudbreakCloudbreak Pharma is a clinical stage ophthalmic drug company with a proprietary technology, Multi Kinase Inhibitors (MKI), that is de-risked with human clinical results and is uniquely suitable for both front-of-eye and back-of-eye diseases. Under the MKI platform, Cloudbreak’s lead asset, the eye drop CBT-001, is in mid-Phase 3 in multiple regions for the treatment of pterygium, a disease impacting 15 million people in the US alone. Its second asset, CBT-004, is entering Phase 3 for pinguecula, a disease impacting about half of all people over age 70. CBT-001 and CBT-004 will potentially be the first and only FDA-approved drug therapies for these high-value conditions. The Company has additional technologies with pre-clinical through Phase 2 product candidates. The Cloudbreak team is comprised of experienced ophthalmologic scientists and business professionals that are uniquely qualified to bring product candidates from pre-clinical to NDA approval. For more information, visit: https://cloudbreakpharma.com/ For an informative video, please click here. Forward Looking Statements This press release contains forward-looking statements regarding the future performance, plans and prospects of Cloudbreak Pharma, Inc. (with effect from 3 July 2025, shares listed on the Main Board of The Stock Exchange of Hong Kong Limited (the “HKEX”)). These statements may include research and development (R&D) activities, clinical trial progress and outcomes, regulatory submissions, timelines and approvals, manufacturing and supply operations, product launches and partnerships and/or financial outlook and business strategy. All forward-looking statements are based on management’s current expectations and assumptions only as of the date hereof. Actual results may differ due to various factors, including clinical or regulatory outcomes (e.g., US or other markets), competitive developments and market dynamics, and/or economic or operational uncertainties. Cloudbreak Pharma, Inc. undertakes no obligation to update or revise any forward-looking statements to reflect subsequent circumstances, except as required by law. The information contained in this presentation may not be complete and may not contain all particulars required to be disclosed by us under the Rules Governing the Listing of Securities on the KHEX and the Securities and Futures Ordinance (Chapter 571 of the Laws of Hong Kong). Investors or interested parties should review our public filings with the HKEX and our company’s website (cloudbreakpharma.com) for additional information. Contacts: For investors:Eric Ribner, LifeSci Advisorseric@lifesciadvisors.com For partnering opportunities:partners@cloudbreakpharma.com

临床2期临床3期临床结果

2026-01-27

·阿米陀陀

《呼吸内科学高级教程》第三十四节结缔组织病所致间质性肺病

一、概述肺间质疾病（ILD）：一组以肺间质炎症和纤维化为特征的疾病，累及肺泡壁、小气道、微血管等。结缔组织病（CTD）是ILD的重要病因之一，占ILD的约15%。自身免疫在ILD发病中起重要作用，部分“特发性”肺纤维化（IPF）患者后期可确诊为CTD。二、流行病学 CTD继发ILD的比例：系统性硬化病（SSc）：60%～100% 多发性肌炎/皮肌炎（PM/DM）：5%～10% 类风湿关节炎（RA）：10%～50% 系统性红斑狼疮（SLE）：3%～13% 原发性舍格伦综合征（pSS）：10% 混合性结缔组织病（MCTD）：30%～85% 病死率较高，尤其是SSc、PM/DM等。北京协和医院数据显示，ILD是CTD常见且严重的并发症。三、病因与发病机制免疫因素是核心，涉及自身抗体（如RF、抗Jo-1、抗Scl-70等）。细胞因子（如TNF-α、TGF-β）和炎症细胞相互作用，促进肺泡炎和纤维化。四、病理类型（参照IIP分类）急性间质性肺炎（AIP）：弥漫性肺泡损伤。寻常型间质性肺炎（UIP）：最常见，易发展为蜂窝肺。非特异性间质性肺炎（NSIP）：弥漫性炎症和纤维化。脱屑性间质性肺炎（DIP）：肺泡内巨噬细胞聚集。淋巴细胞型间质性肺炎（LIP）：多见于pSS。隐原性机化性肺炎（COP/BOOP）：肉芽组织增生，多见于RA、PM/DM。呼吸细支气管炎伴间质性肺炎（RB-ILD）。不同CTD的常见病理类型有所不同，且可重叠。五、临床表现 CTD全身表现：如关节痛、皮疹、肌无力、口干、皮肤硬化等。 ILD相关症状：进行性呼吸困难、干咳肺部可闻及爆裂音（Velcro啰音）晚期可出现杵状指、发绀、肺动脉高压、呼吸衰竭。少数患者以ILD为首发表现，易误诊。六、诊断综合诊断：结合CTD病史、呼吸道症状、体征及辅助检查。关键检查： HRCT：首选，敏感度高于X线。肺功能：限制性通气功能障碍、弥散功能下降。自身抗体检测：如ANA、RF、抗CCP、抗Jo-1等。支气管肺泡灌洗/肺活检：用于疑难病例（金标准）。七、鉴别诊断特发性肺间质纤维化（IPF）：缺乏CTD特征性表现和自身抗体。 CTD合并肺部感染：需通过病原学检查鉴别，尤其是机会性感染（如肺孢子虫）。八、治疗1. 糖皮质激素初始泼尼松0.5～1mg/(kg·d)，逐渐减量。急性期可用甲泼尼龙冲击。2. 免疫抑制剂环磷酰胺：口服或静脉冲击，用于重症或激素无效者。硫唑嘌呤：维持治疗。环孢素A、霉酚酸酯等也可选用。3. 抗纤维化及辅助治疗 N-乙酰半胱氨酸：抗氧化，可能延缓纤维化。秋水仙碱、γ-干扰素、内皮素受体拮抗剂（波生坦）、吡非尼酮等。4. 生物制剂 TNF-α拮抗剂（如英夫利昔单抗）疗效有争议，需个体化评估。5. 支持治疗氧疗、抗感染、支气管扩张剂等。6. 肺移植终末期患者的可选方案。注意事项避免使用可能致肺纤维化的药物（如甲氨蝶呤需谨慎）。治疗需排除感染、药物等其他因素。九、预后与病理类型密切相关： BOOP预后较好，对激素敏感； UIP、DAD预后差，易进展为呼吸衰竭。早期诊断和治疗可改善预后。 ILD是CTD主要死亡原因之一。十、要点总结 CTD-ILD常见且严重，需早期识别。 HRCT和自身抗体检测是关键诊断工具。治疗以激素联合免疫抑制剂为主，需个体化。病理类型影响治疗方案和预后。多学科协作（风湿科、呼吸科）至关重要。副主任医师水平考试模拟试卷：结缔组织病所致间质性肺病（CTD-ILD）一、单选题（共15题，每题1分，占15%）主要考察基础理论、指南共识、疾病诊断标准。关于肺间质疾病（ILD），下列描述正确的是：A. 仅累及肺泡上皮细胞基膜和毛细血管基膜之间的空隙B. 发病率约占呼吸系统疾病的5%以下C. 是一组以肺间质炎症和纤维化为主要特征的疾病，可累及肺泡壁、小气道和微血管D. 所有ILD的预后均很差，治疗手段有限答案：C解析：肺间质疾病（ILD）是一组主要累及肺间质，但同时也可累及肺泡上皮、肺泡腔、小气道和微血管的弥漫性肺部疾病。其发病率约占呼吸系统疾病的15%。ILD病因繁多，预后差异很大，部分类型（如某些CTD-ILD、BOOP）对治疗反应较好。故A、B、D选项均错误。在ILD的病因分类中，结缔组织病（CTD）所占的比例约为：A. 5%B. 10%C. 15%D. 30%答案：C解析：根据文中概述部分，有文献报道，在ILD中15%是由CTD引起。这是重要的流行病学数据。下列哪种结缔组织病（CTD）并发间质性肺病（ILD）的比例最高？A. 类风湿关节炎（RA）B. 系统性红斑狼疮（SLE）C. 系统性硬化病（SSc）D. 原发性舍格伦综合征（pSS）答案：C解析：根据流行病学部分数据，SSc继发ILD的比例高达60%～100%，是所列CTD中比例最高的。RA为10%～50%，SLE为3%～13%，pSS约为10%。与皮肌炎/多肌炎（PM/DM）合并ILD最相关的自身抗体是：A. 抗Scl-70抗体B. 抗Jo-1抗体（抗tRNA合成酶抗体）C. 抗Sm抗体D. 抗CCP抗体答案：B解析：在发病机制部分指出，DM/PM中ILD与抗tRNA合成酶抗体（如Jo-1抗体）有关。抗Scl-70抗体与SSc相关，抗Sm抗体与SLE相关，抗CCP抗体与RA相关。结缔组织病相关间质性肺病（CTD-ILD）最常见的病理类型是：A. 急性间质性肺炎（AIP）B. 寻常型间质性肺炎（UIP）C. 非特异性间质性肺炎（NSIP）D. 淋巴细胞型间质性肺炎（LIP）答案：B解析：病理部分明确指出，UIP是CTD-ILD常见的病理类型。文中列举了各型CTD-ILD的常见病理类型，UIP在RA、SSc、PM/DM、pSS中均易出现。高分辨率CT（HRCT）诊断CTD-ILD的敏感性显著高于胸部X线片，研究显示其检出率可达：A. 约30%B. 约50%C. 约70%D. 约85%以上答案：D解析：影像学部分引用研究显示，81例CTD患者中，69例（85.1%）HRCT示有肺间质病变，而X线仅显示少数。这凸显了HRCT在早期诊断中的核心价值。诊断CTD-ILD的“金标准”是：A. 高分辨率CT（HRCT）B. 肺功能检查C. 自身抗体检测D. 肺活检（经支气管或外科）答案：D解析：诊断部分明确提到，肺活检为诊断ILD的金标准手段。但在临床实践中，对于有典型CTD病史和HRCT表现的患者，并非必须进行有创活检。HRCT是首选的无创重要检查。关于CTD-ILD的肺功能改变，早期最典型的表现是：A. 阻塞性通气功能障碍B. 弥散功能（DLCO）下降和肺容量减少C. 混合性通气功能障碍D. 呼吸肌力减弱答案：B解析：实验室及辅助检查部分指出，肺功能检查早期表现为弥散功能下降和肺容量减少，逐渐发展可出现限制性通气功能障碍。 CTD-ILD患者肺部听诊最具特征性的体征是：A. 哮鸣音B. Velcro啰音（爆裂音）C. 支气管呼吸音D. 胸膜摩擦音答案：B解析：临床表现部分提到，常见体征包括两肺中下部特征性爆裂（Velcro）音。这是ILD相对特异的体征。下列哪种病理类型对糖皮质激素治疗通常反应较好，预后相对较佳？A. 寻常型间质性肺炎（UIP）B. 弥漫性肺泡损伤（DAD）C. 隐原性机化性肺炎（COP/BOOP）D. 急性间质性肺炎（AIP）答案：C解析：预后部分提到，BOOP对激素治疗敏感，有完全缓解倾向，预后较好。而UIP对激素反应差，DAD和AIP通常预后很差。对于CTD-ILD的治疗，目前常推荐的初始治疗方案是：A. 单用大剂量糖皮质激素冲击治疗B. 糖皮质激素联合环磷酰胺或硫唑嘌呤C. 单用抗纤维化药物（如吡非尼酮）D. 单用生物制剂（如TNF-α拮抗剂）答案：B解析：治疗部分明确指出，常推荐的治疗方案是糖皮质激素联合环磷酰胺或硫唑嘌呤。这是基于临床经验的主流方案。首个通过随机双盲安慰剂对照研究证实对特定CTD-ILD（SSc-ILD）有效的药物是：A. 泼尼松B. 环磷酰胺C. 硫唑嘌呤D. N-乙酰半胱氨酸答案：B解析：治疗部分提到，美国多中心研究证实口服环磷酰胺治疗SSc-ILD较安慰剂有统计学意义的改善，是CTD-ILD治疗领域第一个阳性报道的随机双盲对照研究。在治疗CTD-ILD时，通常需避免使用的药物是：A. 羟氯喹B. 环孢素AC. 甲氨蝶呤D. 霉酚酸酯答案：C解析：治疗部分末尾强调，甲氨蝶呤本身可引起肺间质病变，因此对于合并ILD的CTD一般避免使用。但并非绝对禁忌，需权衡利弊。关于N-乙酰半胱氨酸（NAC）在CTD-ILD治疗中的应用，下列哪项是正确的？A. 其疗效已被大规模随机对照试验证实可显著改善CTD-ILD患者预后B. 其作用机制明确为免疫抑制C. 临床上已有较多风湿科医生经验性使用，但尚需更多研究验证其疗效D. 仅适用于IPF，对CTD-ILD无效答案：C解析：治疗部分指出，虽然IPF研究显示大剂量NAC有效，且很多风湿科医生将其用于CTD-ILD，但其对CTD-ILD的疗效还需要进一步的临床研究验证。A、B、D选项表述均不准确。 CTD-ILD终末阶段（蜂窝肺）唯一有效的根治性治疗方法是：A. 大剂量激素冲击治疗B. 长期家庭氧疗C. 肺移植D. 抗纤维化药物持续治疗答案：C解析：治疗部分在肺移植条目下明确指出，对于终末肺（蜂窝肺）阶段惟一有效的治疗方法是肺移植。二、多选题（共10题，每题2分，占20%）难度高于单选，侧重疾病鉴别诊断、并发症、治疗方案的综合选择等核心知识点。下列哪些是结缔组织病（CTD）的共同临床特点？（）A. 常同时或相继出现多个系统或器官受累B. 有一系列自身抗体存在C. 均以肺部间质病变为首发症状D. 病理上均有血管炎表现E. 治疗均以糖皮质激素为基础答案：ABE解析：临床表现部分指出CTD的两个主要特点为多系统受累和自身抗体存在。治疗部分指出糖皮质激素为最常用药。C选项错误，仅少数以ILD为首发。D选项错误，并非所有CTD都有血管炎。下列自身抗体与对应的CTD-ILD风险关联正确的是？（）A. 抗Jo-1抗体 —— 皮肌炎/多肌炎（PM/DM）相关ILDB. 抗Scl-70抗体 —— 系统性硬化病（SSc）相关ILDC. 高滴度类风湿因子（RF）—— 类风湿关节炎（RA）相关ILD风险增高D. 抗SSA/SSB抗体 —— 舍格伦综合征（pSS）相关ILDE. 抗ds-DNA抗体 —— 提示SLE活动，但与ILD发生无关答案：ABCD解析：病因和发病机制部分提到了A、B、C选项的关联。实验室检查部分提到了D选项。虽然抗ds-DNA抗体主要与SLE肾脏受累等相关，但SLE本身可累及肺，且文中SLE部分未特指某抗体与ILD强相关，E选项“无关”表述过于绝对，且不符合SLE为全身性疾病的特点，故不选。关于CTD-ILD的病理学，以下描述正确的有？（）A. 病理类型与非结缔组织病相关的特发性间质性肺炎（IIP）类型相似B. 同一患者的肺组织内，不同病理类型可重叠存在C. 非特异性间质性肺炎（NSIP）在SSc、PM/DM、RA等多种CTD中均易出现D. 淋巴细胞型间质性肺炎（LIP）几乎仅见于系统性红斑狼疮（SLE）E. 寻常型间质性肺炎（UIP）对糖皮质激素治疗反应通常良好答案：ABC解析：病理部分提到CTD-ILD病理与IIP相似（A）。同一病例可有重叠现象（B）。NSIP易在多种CTD中出现（C）。LIP多见于pSS，但也可见于SLE、MCTD（D错误）。UIP对激素反应差（E错误）。高分辨率CT（HRCT）上，CTD-ILD可能出现的表现包括？（）A. 磨玻璃样影B. 蜂窝状阴影C. 小叶间隔增厚D. 牵拉性支气管扩张E. 胸膜下线答案：ABCDE解析：影像学部分详细列出了HRCT的九种主要表现，包括了所有选项内容。需要与CTD-ILD进行鉴别诊断的疾病主要包括？（）A. 特发性肺间质纤维化（IPF）B. CTD合并肺部感染（尤其是机会性感染）C. 药物性肺损伤（如甲氨蝶呤所致）D. 心源性肺水肿E. 肺泡蛋白沉积症答案：ABC解析：鉴别诊断部分明确列出了IPF和CTD合并感染（特别是卡氏肺孢子虫）。治疗部分特别提到需排除药物（如甲氨蝶呤）引起的肺损伤。D和E虽然是弥漫性肺病，但文中未作为重点鉴别列出，且临床特征差异较大，故根据原文重点，答案选ABC。关于CTD-ILD的糖皮质激素治疗，下列观点正确的有？（）A. 是治疗CTD-ILD的最常用药物B. 推荐起始剂量泼尼松0.5～1mg/(kg·d)，维持6～8周后逐渐减量C. 对于HRCT显示大片磨玻璃影的急性病变，可考虑甲泼尼龙冲击治疗D. 所有病理类型的CTD-ILD对糖皮质激素均有良好反应E. 疗效与肺纤维化程度密切相关，晚期纤维化者疗效差答案：ABCE解析：治疗部分支持A、B、C选项。同时指出糖皮质激素的反应取决于纤维化进展的程度及病理类型，UIP反应不佳，晚期效果差。故D错误，E正确。可用于治疗CTD-ILD的免疫抑制剂包括？（）A. 环磷酰胺B. 硫唑嘌呤C. 环孢素AD. 霉酚酸酯E. 他克莫司答案：ABCDE解析：治疗部分提到了环磷酰胺、硫唑嘌呤、环孢素A、羟氯喹。霉酚酸酯和他克莫司虽未在原文明确列出，但属于临床常用的免疫抑制剂，且在风湿病治疗中应用广泛，故根据临床实践，所有选项均可选。本题考察知识迁移能力。下列哪些药物或方法被认为具有抗肺纤维化作用，可能用于CTD-ILD的治疗？（）A. 秋水仙碱B. γ-干扰素C. 内皮素受体拮抗剂（波生坦）D. 吡非尼酮E. N-乙酰半胱氨酸（NAC）答案：ABCDE解析：治疗部分的“抗纤维化药物”和“N-乙酰半胱氨酸”小节，明确提到了所有选项的药物及其潜在作用机制。关于生物制剂（如TNF-α拮抗剂）治疗CTD-ILD，以下描述正确的有？（）A. 是治疗RA-ILD的一线推荐药物B. 有研究报道其可能使已有的ILD加重C. 也有研究报道其可能改善RA相关的肺部病变D. 目前疗效尚不确切，仍在观察中E. 在任何CTD-ILD中均应禁止使用答案：BCD解析：治疗部分指出，关于TNF-α拮抗剂治疗RA-ILD，有报道称其可加重ILD，也有报道称其减轻病变，因此疗效仍在观察中。A、E选项表述绝对且错误。影响CTD-ILD预后的因素包括？（）A. 基础CTD的类型B. ILD的病理类型C. 诊断和治疗的时机D. 是否合并肺动脉高压E. 对治疗的反应答案：ABCDE解析：全文综合判断。流行病学显示不同CTD的ILD发生率和病死率不同（A）。预后部分强调病理类型与预后关系大（B）。概述和治疗部分强调早期诊断治疗的重要性（C）。临床表现和预后部分提到可合并肺动脉高压，是严重并发症（D）。治疗反应直接影响预后（E）。三、共用题干单选题（A3/A4型题）（共3个案例，每个案例下设3-5问，共15问，每题1分，占15%）以临床真实病例为题干，考察从病史、检查到诊断、治疗、预后的全流程思维。【案例一】患者，女性，52岁，因“进行性呼吸困难、干咳6个月”入院。近2个月出现双手指遇冷变白、变紫。体检：T 36.5℃，P 96次/分，R 24次/分，BP 120/80mmHg。呼吸稍促，双肺下野可闻及Velcro啰音。双手指皮肤变硬、紧绷，面部皱纹减少，张口度减小。为明确诊断，首选的影像学检查是：A. 胸部正侧位X线片B. 胸部高分辨率CT（HRCT）C. 肺部增强CTD. 肺通气/灌注显像答案：B解析：患者有进行性呼吸困难、干咳、Velcro啰音，高度怀疑ILD。HRCT是诊断ILD最敏感、最首选的无创影像学检查，能清晰显示间质性病变的细节。该患者最可能的基础结缔组织病是：A. 类风湿关节炎B. 系统性红斑狼疮C. 系统性硬化病D. 皮肌炎答案：C解析：患者有典型的雷诺现象，以及手指、面部皮肤硬化、紧绷，张口受限，是系统性硬化病（SSc）的特征性表现。为进一步支持SSc的诊断，应重点检查的自身抗体是：A. 抗Jo-1抗体B. 抗Scl-70抗体C. 抗Sm抗体D. 抗CCP抗体答案：B解析：抗Scl-70抗体是SSc的标志性抗体，与弥漫性皮肤受累和ILD发生风险增高相关。（假设HRCT提示双下肺网格状影、牵拉性支气管扩张，少量磨玻璃影）该患者ILD最可能的病理类型是：A. 淋巴细胞型间质性肺炎（LIP）B. 隐原性机化性肺炎（COP）C. 寻常型间质性肺炎（UIP）D. 急性间质性肺炎（AIP）答案：C解析：根据描述，HRCT表现符合UIP的特征（网格状影、牵拉性支扩，磨玻璃影少）。且文中病理部分指出UIP是CTD-ILD常见类型，易在SSc中出现。该患者ILD的治疗，目前有随机对照研究证据支持的药物是：A. 单用大剂量泼尼松B. 环磷酰胺C. 吡非尼酮D. 英夫利西单抗答案：B解析：治疗部分指出，针对SSc-ILD，环磷酰胺是首个在随机双盲安慰剂对照研究中显示有效的药物。【案例二】患者，男性，48岁，因“对称性多关节肿痛3年，加重伴气短1个月”就诊。关节痛以双手掌指、近端指间关节及双腕为主，有晨僵。1月来上楼即感气短，干咳。体检：双手指尺侧偏斜，双腕肿胀。双肺底可闻及少量细湿啰音。该患者最可能的基础疾病是：A. 骨关节炎B. 痛风C. 类风湿关节炎D. 强直性脊柱炎答案：C解析：对称性小关节肿痛、晨僵、关节畸形，符合类风湿关节炎（RA）的典型表现。为评估其肺部情况，除HRCT外，最有价值的检查是：A. 肺功能检查（包括弥散功能）B. 支气管镜检查C. 痰找抗酸杆菌D. 动脉血气分析答案：A解析：肺功能检查，特别是弥散功能（DLCO），是评估ILD严重程度和监测病情变化的客观指标。血气分析在静息状态下可能正常，运动后或晚期才有异常。若该患者确诊为RA-ILD，在制定治疗方案时，需特别注意：A. 立即加用生物制剂B. 避免使用甲氨蝶呤C. 加大非甾体抗炎药剂量D. 仅使用抗纤维化药物答案：B解析：治疗部分强调，甲氨蝶呤本身可引起肺损伤，对于合并ILD的CTD（尤其是RA）一般避免使用。如果患者经糖皮质激素联合环磷酰胺治疗后，关节症状控制不佳，但ILD稳定，考虑调整风湿病情用药。下列哪种生物制剂的使用需格外谨慎？A. 阿达木单抗（TNF-α拮抗剂）B. 利妥昔单抗（抗CD20单抗）C. 托珠单抗（IL-6受体拮抗剂）D. 阿巴西普（CTLA4-Ig细胞毒性 T 淋巴细胞相关抗原 4）答案：A解析：治疗部分指出，TNF-α拮抗剂治疗RA-ILD的疗效存在争议，且有报道可能加重ILD，因此使用时需谨慎评估和密切监测。【案例三】患者，女性，35岁，因“发热、肌肉疼痛、乏力2周，突发呼吸困难2天”急诊入院。查体：T 38.8℃，R 32次/分，SpO2 88%（吸空气）。双肺可闻及广泛湿啰音。四肢近端肌肉压痛，肌力IV级。面部及前胸可见红色皮疹。该患者最紧急的处理措施是：A. 立即行支气管镜检查B. 急诊肺功能检查C. 给予高流量氧疗，必要时机械通气D. 立即肌肉活检答案：C解析：患者急性起病，严重低氧血症，呼吸衰竭是当前主要矛盾，必须首先稳定生命体征，给予积极氧疗或呼吸支持。结合临床表现，该患者最可能的诊断是：A. 社区获得性肺炎B. 皮肌炎合并快速进展性间质性肺病C. 重症哮喘急性发作D. 急性肺栓塞答案：B解析：患者有发热、肌痛、肌无力、特征性皮疹（皮肌炎表现），同时突发严重呼吸困难和低氧血症，双肺湿啰音，高度提示皮肌炎（DM）合并急性或快速进展性间质性肺病，可能为病理上的弥漫性肺泡损伤（DAD）或急性间质性肺炎（AIP）。为明确肺部病变性质，在病情允许的情况下，最具诊断价值的检查是：A. 查血肌酶谱B. 查抗Jo-1抗体C. 急诊胸部HRCTD. 经支气管镜肺活检（TBLB）答案：C解析： HRCT能快速无创地显示肺部病变的范围和性质（如弥漫性磨玻璃影、实变，提示急性肺泡损伤），对紧急决策至关重要。抗Jo-1抗体和肌酶有助于诊断皮肌炎，但不能立即明确肺部急症的性质。TBLB有创，在急重症患者中风险较高，非首选。该患者肺部病变的病理类型最可能是：A. 非特异性间质性肺炎（NSIP）B. 寻常型间质性肺炎（UIP）C. 弥漫性肺泡损伤（DAD）D. 闭塞性细支气管炎伴机化性肺炎（BOOP）答案：C解析：急性起病、快速进展的严重呼吸衰竭，在DM/PM中常对应病理类型为弥漫性肺泡损伤（DAD），预后极差。初始药物治疗方案应首选：A. 大剂量糖皮质激素冲击治疗（如甲泼尼龙）B. 口服小剂量泼尼松C. 单用环磷酰胺D. 抗感染治疗答案：A解析：对于临床上表现为急性、重症的肺间质病变，治疗部分指出可考虑大剂量甲泼尼龙冲击治疗，以期快速抑制免疫炎症反应。四、案例分析题（共1个案例，下设8-10问，共30分，占30%）题干为复杂疑难病例，问题涵盖诊断、鉴别诊断、检查、治疗、监测、预后等。【案例】患者，女性，60岁，因“反复关节痛、口干、眼干10余年，咳嗽、进行性呼吸困难2年，加重1周”入院。现病史： 10余年前无诱因出现双手腕、膝关节对称性肿痛，伴晨僵，间断口服“止痛药”治疗。同时有口干、需频频饮水，眼干、异物感。2年前开始出现间断干咳，活动后气短，并逐渐加重，上2层楼即需休息。1周前受凉后咳嗽加重，咳少量白痰，气短明显，静息时亦感呼吸困难，伴发热，体温最高38.5℃。既往史：无特殊。查体： T 38.0℃，P 110次/分，R 28次/分，BP 130/80mmHg。神清，呼吸急促，口唇轻度发绀。双肺呼吸音粗，双下肺可闻及密集的Velcro啰音。心脏、腹部查体未见异常。双手腕关节轻度肿胀，无畸形。辅助检查（外院）：血常规：WBC 12.5×10⁹/L，N 85%。ESR 65mm/h，CRP 48mg/L。胸部X线片：双肺纹理增粗、紊乱，双下肺可见网格状影。问题：该患者的初步诊断应考虑哪些可能？（至少列出3个）为明确诊断，应立即安排哪些关键检查？（至少列出4项）患者目前气短加重，可能的原因是什么？如何鉴别？若该患者自身抗体谱回报：ANA 1:320（颗粒型），抗SSA抗体阳性，抗SSB抗体阳性，RF 120 IU/mL。高分辨率CT（HRCT）显示：双肺弥漫性磨玻璃影，伴小叶间隔增厚，双下肺为主，可见少量细小囊状影。肺功能提示：限制性通气功能障碍，DLCO占预计值45%。请给出完整的诊断。该患者CTD-ILD最可能的病理类型是什么？为什么？请制定该患者当前急性加重期的治疗方案。如果初始治疗（糖皮质激素联合免疫抑制剂）效果不佳，可考虑哪些二线或辅助治疗选择？在治疗过程中，需要重点监测哪些指标以评估疗效和不良反应？该患者本次急性加重的可能诱因是什么？对预后有何提示？长期管理中，应告知患者注意哪些事项？【答案与解析】初步诊断考虑：结缔组织病（CTD）相关间质性肺病（CTD-ILD）急性加重：患者有长期关节痛、口眼干等CTD症状，近期ILD症状（咳嗽、呼吸困难）急性加重，符合此诊断思路。 CTD合并社区获得性肺炎（CAP）：患者有受凉史，发热，咳痰，血象及炎性指标升高，需警惕在原有肺间质病基础上合并感染。特发性肺间质纤维化（IPF）急性加重：虽然患者有CTD症状，但若CTD诊断不明确，需考虑IPF。但IPF急性加重常无明确感染诱因。（可能的CTD类型：类风湿关节炎、舍格伦综合征或重叠综合征）。应立即安排的关键检查：胸部高分辨率CT（HRCT）：评估ILD的当前范围、性质（磨玻璃影、实变、纤维化）及有无感染征象。动脉血气分析：评估缺氧和呼吸衰竭的严重程度。自身抗体谱：包括ANA、ENA谱（抗SSA、SSB、RNP、Scl-70、Jo-1等）、RF、抗CCP抗体，以明确CTD类型。病原学检查：痰涂片、痰培养+药敏、血培养、必要时查G试验/GM试验、呼吸道病原体核酸，以鉴别或排除感染。（可选）支气管肺泡灌洗（BAL）：在感染鉴别困难时，可行BAL进行细胞分类和病原学检查。气短加重的原因及鉴别：原因： ① CTD-ILD本身急性进展； ② 合并肺部感染（细菌、病毒、真菌或机会性病原体如肺孢子菌）； ③ 肺栓塞； ④ 心功能不全。鉴别：感染 vs. 疾病活动：感染常有明确诱因、发热、脓痰、血象和CRP显著升高，影像学出现新的实变或磨玻璃影。疾病活动可能炎性指标也升高，但病原学阴性。肺栓塞：突发胸痛、咯血、低氧血症，D-二聚体升高，CTPA可确诊。心衰：端坐呼吸、咳粉红色泡沫痰、肺底湿啰音、心脏扩大、BNP升高。完整诊断：原发性舍格伦综合征（pSS）舍格伦综合征相关间质性肺病（pSS-ILD）（根据HRCT表现，可能为淋巴细胞型间质性肺炎LIP或非特异性间质性肺炎NSIP）肺部感染（社区获得性肺炎？待病原学确认） I型呼吸衰竭最可能的病理类型及原因：最可能：淋巴细胞型间质性肺炎（LIP）。原因： ① 文中病理部分指出，LIP多见于舍格伦综合征（pSS）。② HRCT表现（弥漫性磨玻璃影、小叶间隔增厚、细小囊状影）与文中描述的LIP的HRCT特点（磨玻璃影、广泛囊状影）相符。急性加重期治疗方案：抗感染治疗：根据病原学结果或经验性覆盖社区常见病原体及可能的机遇菌（如覆盖肺孢子菌）。呼吸支持：根据血气结果给予氧疗，维持SpO2>90%。免疫抑制治疗：在强烈怀疑感染已控制或排除主要感染后，可考虑使用糖皮质激素控制ILD活动。可起始静脉甲泼尼龙40-80mg/d或等效剂量，根据反应调整。对症支持治疗：化痰、营养支持等。二线或辅助治疗选择：更换或联合免疫抑制剂：如环磷酰胺、霉酚酸酯、环孢素A、他克莫司等。生物制剂：如利妥昔单抗（抗CD20单抗）对部分pSS及pSS-ILD有效。抗纤维化药物：如吡非尼酮、尼达尼布（需更多CTD-ILD证据）。辅助治疗： N-乙酰半胱氨酸（NAC）。监测指标：疗效监测：症状（呼吸困难评分）、体征（肺部啰音）、静息及活动后SpO2、动脉血气、HRCT（变化较慢）、肺功能（特别是DLCO，每3-6月）。不良反应监测：血常规、肝肾功能、电解质、血糖（激素相关）；感染迹象；免疫抑制剂特定毒性（如环磷酰胺的出血性膀胱炎、骨髓抑制；环孢素A的肾毒性、高血压）。急性加重诱因及预后提示：可能诱因：肺部感染是导致CTD-ILD急性加重的最常见诱因。预后提示：发生急性加重通常提示预后较差，尤其是出现呼吸衰竭者。需要积极寻找并处理诱因，同时强化免疫抑制治疗。长期管理注意事项：定期随访：风湿免疫科和呼吸科联合随访。坚持治疗：遵医嘱规律用药，不自行减停。预防感染：接种流感疫苗、肺炎球菌疫苗；避免去人群密集场所；注意个人卫生。康复锻炼：在医生指导下进行肺康复训练。戒烟并避免吸入刺激物。报告新症状：如出现发热、咳嗽咳痰加重、呼吸困难加剧、咯血等，需立即就医。监测并发症：注意有无肺动脉高压、肺心病迹象。

临床研究临床结果申请上市

2026-01-27

·临床研究促进公益基金

从参与者到引领者：中国医药的创新转型与价值跃迁｜要闻

2025年全年共有76个创新药获批上市，数量较上年增长58%；中国创新药对外授权交易总金额首次突破1300亿美元，交易笔数超过150笔，标志着中国正从全球制药产业链的参与者，向创新策源地和价值输出者加速转型。撰文｜毛冬蕾国家药品监督管理局（NMPA）近日公布的年度数据显示，2025年全年共有76个创新药获批上市，数量较上年增长58%；与此同时，中国创新药对外授权交易总金额首次突破1300亿美元，交易笔数超过150笔。本土创新引领产业质变全年批准的11个全新治疗机制的药物中，有4个源自中国企业的自主研发。2025年国家药监局批准上市的76个创新药包括47个化学药品、23个生物制品和6个中药。47个化学药品中，38个为国产创新药，9个为进口创新药，国产创新药占比达80.85%；23个生物制品中，21个为国产创新药，2个为进口创新药，国产创新药占比达91.30%。 AI制作本土全球化企业展现了覆盖全链条的体系化创新能力。例如，百济神州获批国内首款治疗HER2高表达胆道癌的HER2双特异性抗体；恒瑞医药的注射用瑞康曲妥珠单抗成为国内首款治疗HER2突变肺癌的自主研发ADC药物；细胞治疗、靶向蛋白降解等前沿技术也从实验室加速走向临床。而正大天晴的库莫西利胶囊作为全球首款CDK2/4/6抑制剂，为乳腺癌耐药患者提供新方案；和美药业的莫米司特片作为我国首个自主研发的PDE4抑制剂，改写了中重度银屑病的治疗格局；泽璟生物的盐酸吉卡昔替尼片成为首个国产骨髓纤维化JAK抑制剂。恒瑞医药的苹果酸法米替尼胶囊为晚期宫颈癌患者提供了新的靶免联合疗法；其全氟己基辛烷滴眼液成为全球唯一治疗睑板腺功能障碍相关干眼的药品；瑞格列汀二甲双胍片则是国内首款自主研发的DPP-4抑制剂类降糖复方制剂。这些新药代表了创新型药企集群的崛起，共同构成了支撑千亿美元级对外授权的基石。前沿疗法加速引入中国 2025年，中国市场与国际创新的同步性达到新高度。多家跨国制药公司的新药及新适应症获批上市，为多个疾病领域的患者提供了创新治疗方案。其中，勃林格殷格翰的维加特®（通用名：尼达尼布）在年初获批新增进行性纤维化性间质性肺疾病（PF-ILD）适应症，使其治疗范围从特发性肺纤维化（IPF）扩展至更广泛的肺纤维化患者群体。与此同时，其他跨国药企也带来了多项“首个”突破：强生公司的古塞奇尤单抗成为国内首个IL-23抑制剂，先后获批用于克罗恩病和溃疡性结肠炎；辉瑞的阿昔替尼联合疗法是国内首个用于晚期肾细胞癌的特定靶免联合方案，其新型抗菌药物氨曲南阿维巴坦钠也成功获批；诺华推出了全球首个基于ABL肉豆蔻酰口袋（STAMP）的变构抑制剂阿思尼布，用于新诊断的Ph+慢性髓细胞白血病；赛诺菲的肥厚型心肌病新药阿夫凯泰片更是实现了“中美双报，中国首发”。此外，罗氏利司扑兰的新片剂剂型、诺和诺德司美格鲁肽的心血管新适应症，以及默克用于治疗症状性腱鞘巨细胞瘤的盐酸匹米替尼，均在2025年内获得批准。这些获批项目涵盖了自身免疫疾病、肿瘤、抗感染、心血管、罕见病等多个关键治疗领域。这些药物的快速获批，得益于中国持续深化的药品审评审批制度改革。“突破性治疗药物程序、附条件批准、优先审评审批、特别审批”四大快速通道的高效运转，在坚持国际标准的同时，大幅压缩了审评时限，确保了全球前沿疗法能与中国患者“零时差”相遇。展望未来，国家药监局表示将进一步优化审评资源，向新机制、新靶点药物倾斜，并通过完善药品试验数据保护与市场独占期制度，系统强化对创新的知识产权保护。可以预见，在政策、资本与人才的驱动下，中国创新药企将加大源头创新，而跨国企业也将更融入本土研发生态。中国生物医药产业，正以其创新实力和开放的姿态，迎接从“中国新”迈向“全球新”的机遇。（数据及部分素材来源于国家药监局微信公众号）公众健康离不开医学进步医学进步离不开临床研究我们离不开公众的理解支持

上市批准抗体药物偶联物引进/卖出细胞疗法免疫疗法

100 项与乙磺酸尼达尼布相关的药物交易

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外链

KEGG	Wiki	ATC	Drug Bank
D10396 D10481	乙磺酸尼达尼布	L01XE31	DB09079

研发状态

批准上市

10 条最早获批的记录,

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适应症	国家/地区	公司	日期
局部晚期非小细胞肺癌	澳大利亚	Boehringer Ingelheim Pty Ltd.	2015-09-01
转移性非小细胞肺癌	澳大利亚	Boehringer Ingelheim Pty Ltd.	2015-09-01
复发性非小细胞肺癌	澳大利亚	Boehringer Ingelheim Pty Ltd.	2015-09-01
系统性硬化相关的间质性肺病	欧盟	Boehringer Ingelheim International GmbH	2015-01-14
系统性硬化相关的间质性肺病	冰岛	Boehringer Ingelheim International GmbH	2015-01-14
系统性硬化相关的间质性肺病	列支敦士登	Boehringer Ingelheim International GmbH	2015-01-14
系统性硬化相关的间质性肺病	挪威	Boehringer Ingelheim International GmbH	2015-01-14
进行性纤维化间质性肺病	欧盟	Boehringer Ingelheim International GmbH	2015-01-14
进行性纤维化间质性肺病	冰岛	Boehringer Ingelheim International GmbH	2015-01-14
进行性纤维化间质性肺病	列支敦士登	Boehringer Ingelheim International GmbH	2015-01-14
进行性纤维化间质性肺病	挪威	Boehringer Ingelheim International GmbH	2015-01-14
肺腺癌	欧盟	Boehringer Ingelheim International GmbH	2014-11-21
肺腺癌	冰岛	Boehringer Ingelheim International GmbH	2014-11-21
肺腺癌	列支敦士登	Boehringer Ingelheim International GmbH	2014-11-21
肺腺癌	挪威	Boehringer Ingelheim International GmbH	2014-11-21
特发性肺纤维化	美国	Boehringer Ingelheim Pharmaceuticals, Inc.	2014-10-15

未上市

10 条进展最快的记录,

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适应症	最高研发状态	国家/地区	公司	日期
慢性间质性肺病	申请上市	欧盟	Accord Healthcare SLU	2024-02-22
间质性肺疾病	申请上市	美国	Boehringer Ingelheim GmbH	2023-07-25
翼状胬肉	临床3期	美国	Cloudbreak Therapeutics LLC	2022-06-30
翼状胬肉	临床3期	中国	Cloudbreak Therapeutics LLC	2022-06-30
翼状胬肉	临床3期	澳大利亚	Cloudbreak Therapeutics LLC	2022-06-30
翼状胬肉	临床3期	印度	Cloudbreak Therapeutics LLC	2022-06-30
翼状胬肉	临床3期	新西兰	Cloudbreak Therapeutics LLC	2022-06-30
硬皮病	临床3期	美国	Boehringer Ingelheim International GmbH	2017-12-05
硬皮病	临床3期	日本	Boehringer Ingelheim International GmbH	2017-12-05
硬皮病	临床3期	阿根廷	Boehringer Ingelheim International GmbH	2017-12-05

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临床结果

适应症

分期

评价

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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


NCT06070610 (CTgov)	临床1期	-	14	Pirfenidone+Nintedanib (Pirfenidone/Nintedanib alone (Reference))	憲觸襯鹹襯鏇膚獵鹹範(範齋壓廠鹹淵窪廠壓膚) = 膚蓋繭選簾廠夢衊築艱鹽觸構顧窪繭構蓋艱鑰 (膚夢顧網襯憲壓願壓糧, NA) 更多	-	2025-12-02
				Pirfenidone+Nerandomilast+Nintedanib (Pirfenidone/Nintedanib in combination with Nerandomilast (Test))	憲觸襯鹹襯鏇膚獵鹹範(範齋壓廠鹹淵窪廠壓膚) = 鏇遞壓窪夢製窪築獵蓋鹽觸構顧窪繭構蓋艱鑰 (膚夢顧網襯憲壓願壓糧, NA) 更多
NCT03820726 (INBUILD-ON, Pubmed \| Lung)	临床3期	进行性纤维化间质性肺病	-	Continued Nintedanib	衊鑰選鬱齋廠憲網憲壓(選繭簾襯製艱簾簾膚遞) = Diarrhoea was the most frequent adverse event. Amongst patients who had diarrhoea, 90.0% experienced only events of mild or moderate severity 衊醖鹹廠願廠膚憲觸糧 (製獵顧獵繭蓋鬱築鹹選 ) 更多	积极	2025-12-01
ACR2025	N/A	系统性硬化相关的间质性肺病	63	Nintedanib (SSc-ILD)	簾簾壓餘衊獵鹽衊夢蓋(廠鑰膚衊獵壓膚積積選) = 範膚膚獵範鹽憲齋衊窪齋醖鹹築廠選繭窪壓鹽 (壓齋製襯製遞範簾繭築 ) 更多	积极	2025-10-24
ACR2025	N/A	原发性干燥综合征 anti-SSA/Ro \| anti-SSB/La	45	Glucocorticoids	範鹽壓鏇衊淵餘淵獵餘(醖壓齋築壓積窪願鹽積) = 衊壓憲衊顧網憲網願衊壓廠夢積獵鹽觸壓醖膚 (選繭餘餘淵築獵鑰糧範, 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)	遞網構醖艱襯積膚淵衊(觸淵壓願構齋膚蓋襯膚) = 顧觸廠夢淵糧繭蓋構醖餘壓壓醖積衊憲憲築壓 (鹽範壓餘構鑰構餘鹽夢 ) 更多	积极	2025-10-24
				pirfenidone (RA-ILD + SSc-ILD + SADs-ILD)	遞網構醖艱襯積膚淵衊(觸淵壓願構齋膚蓋襯膚) = 壓壓糧醖顧鑰獵艱淵憲餘壓壓醖積衊憲憲築壓 (鹽範壓餘構鑰構餘鹽夢 ) 更多
ACR2025	N/A	类风湿性关节炎伴有类风湿性肺病	74	Nintedanib combined with bDMARDs	壓鏇壓夢範願襯襯糧鏇(願衊網範憲膚艱醖繭廠) = no significant decline 簾鹽艱積範觸鏇選範夢 (醖鑰顧衊鏇窪鬱願醖鏇 ) 更多	积极	2025-10-24
				Nintedanib combined with sDMARDs
ACR2025	N/A	进展性肺纤维化	333	Nintedanib	鏇繭願餘製願膚鬱艱鹽(網範顧網鹹餘鹽糧製製) = 築鬱廠膚蓋憲製壓鹽憲選願糧鬱製繭觸繭膚鬱 (蓋顧壓鹹鏇繭窪醖願鹽 ) 更多	积极	2025-10-24
PACIFIC (ESMO2025)	临床3期	非小细胞肺癌 III期	895	nintedanib	製鑰遞糧願鹹願繭鏇鑰(壓廠齋製憲蓋遞壓糧窪) = 窪窪壓鏇鹽醖願築壓醖壓糧願網鬱憲鏇鏇壓觸 (夢構構窪遞築襯積艱膚 ) 更多	积极	2025-10-17
				osimertinib	製鑰遞糧願鹹願繭鏇鑰(壓廠齋製憲蓋遞壓糧窪) = 鹹憲範鹹壓製壓醖製壓壓糧願網鬱憲鏇鏇壓觸 (夢構構窪遞築襯積艱膚 ) 更多
EULAR2025	N/A	间质性肺疾病	74	Nintedanib + bDMARDs	憲積築衊鬱壓鹽膚糧鑰(遞網繭製積齋鹹製艱餘) = no significant decline 繭糧選窪顧艱顧憲憲艱 (網蓋衊壓築製膚鏇糧築 ) 更多	积极	2025-06-11
				Nintedanib + sDMARDs
NCT05065190 (CTgov)	临床3期	进行性纤维化间质性肺病	81	Placebo (Placebo)	簾範鬱憲蓋積鏇襯構壓 = 鏇簾糧艱襯窪願遞餘繭選餘醖鏇齋願築窪鹹糧 (廠範襯顧窪築簾鹽製網, 窪觸鏇製鏇壓淵鬱鏇遞 ~ 壓繭憲選齋襯繭範遞觸) 更多	-	2025-05-20
				Nintedanib (150 mg Nintedanib)	簾範鬱憲蓋積鏇襯構壓 = 積廠顧簾糧蓋醖襯廠顧選餘醖鏇齋願築窪鹹糧 (廠範襯顧窪築簾鹽製網, 網繭選醖憲鹹淵構鏇積 ~ 淵蓋製憲醖壓衊遞夢鹹) 更多