Idiopathic Pulmonary Fibrosis - Pipeline Insight, 2026

DelveInsight's, "Idiopathic Pulmonary Fibrosis - Pipeline Insight, 2026" report provides comprehensive insights about 100+ companies and 100+ pipeline drugs in Idiopathic Pulmonary Fibrosis pipeline landscape. It covers the pipeline drug profiles, including clinical and nonclinical stage products. It also covers the therapeutics assessment by product type, stage, route of administration, and molecule type. It further highlights the inactive pipeline products in this space.

Geography Covered:

• Global coverage

Idiopathic Pulmonary Fibrosis: Understanding

Idiopathic Pulmonary Fibrosis: Overview

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterized by irreversible scarring and fibrosis of the lung tissue without an identifiable cause. It predominantly affects older adults and commonly presents with gradually worsening exertional dyspnea, a persistent dry cough, and fatigue. Pulmonary function testing typically demonstrates a restrictive ventilatory defect with reduced forced vital capacity (FVC) and impaired diffusing capacity for carbon monoxide (DLCO). IPF has a progressive course and can ultimately lead to respiratory failure and death.

The exact cause of IPF remains unknown, although the disease is believed to result from interactions between genetic susceptibility, aging, and environmental or occupational exposures. Repeated injury to the alveolar epithelium associated with cigarette smoking, air pollution, respiratory infections, chronic aspiration, and other inhaled exposures may contribute to disease development. Advanced age is a major risk factor, while familial cases have been associated with mutations in genes involved in telomere maintenance and surfactant production, including TERT, SFTPA2, and MUC5B. A history of exposure to dust, metals, asbestos, mold, and birds should also be assessed when evaluating patients because these exposures can cause other interstitial lung diseases that need to be excluded before diagnosing IPF.

The pathophysiology of IPF is thought to begin with repeated injury to alveolar epithelial cells, followed by an abnormal and dysregulated repair response. Epithelial injury activates signaling pathways that promote recruitment and proliferation of fibroblasts and differentiation into myofibroblasts. These cells produce excessive extracellular matrix, resulting in progressive deposition of collagen and other matrix components within the lung interstitium. Pro-fibrotic mediators, particularly transforming growth factor-beta (TGF-B), together with other cytokines and signaling pathways involving WNT, Notch, and sonic hedgehog, contribute to persistent fibroblast activation and tissue remodeling. Over time, fibrosis distorts and destroys normal lung architecture, producing the characteristic usual interstitial pneumonia (UIP) pattern with subpleural fibrosis and honeycombing. This structural damage reduces lung compliance and impairs gas exchange, ultimately causing progressive hypoxemia and respiratory failure.

Diagnosis of IPF requires a combination of clinical assessment, exclusion of alternative causes of interstitial lung disease, pulmonary function testing, and high-resolution computed tomography (HRCT). Patients commonly present with progressive exertional dyspnea and a dry cough, while physical examination may reveal fine bibasilar "Velcro-like" crackles and, in some cases, digital clubbing. PFTs generally show a restrictive pattern with reduced lung volumes, particularly FVC and total lung capacity, along with decreased DLCO. HRCT is central to diagnosis and typically demonstrates a UIP pattern characterized by bilateral, predominantly basal and peripheral reticulation, architectural distortion, and honeycombing.

Treatment of IPF focuses on slowing disease progression, managing symptoms and complications, and maintaining quality of life. The approved antifibrotic therapies pirfenidone and nintedanib can slow the decline in lung function and are recommended as disease-modifying treatments. Supportive management includes smoking cessation, supplemental oxygen for patients with hypoxemia, pulmonary rehabilitation, vaccination, and management of comorbidities such as gastroesophageal reflux disease and pulmonary hypertension.

"Idiopathic Pulmonary Fibrosis- Pipeline Insight, 2026" report by DelveInsight outlays comprehensive insights of present scenario and growth prospects across the indication. A detailed picture of the Idiopathic Pulmonary Fibrosis pipeline landscape is provided which includes the disease overview and Idiopathic Pulmonary Fibrosis treatment guidelines. The assessment part of the report embraces, in depth Idiopathic Pulmonary Fibrosis commercial assessment and clinical assessment of the pipeline products under development. In the report, detailed description of the drug is given which includes mechanism of action of the drug, clinical studies, NDA approvals (if any), and product development activities comprising the technology, Idiopathic Pulmonary Fibrosis collaborations, licensing, mergers and acquisition, funding, designations and other product related details.

Report Highlights:

• The companies and academics are working to assess challenges and seek opportunities that could influence Idiopathic Pulmonary Fibrosis R&D. The therapies under development are focused on novel approaches to treat/improve Idiopathic Pulmonary Fibrosis.

Idiopathic Pulmonary Fibrosis Emerging Drugs Chapters

This segment of the Idiopathic Pulmonary Fibrosis report encloses its detailed analysis of various drugs in different stages of clinical development, including phase II, I, preclinical and Discovery. It also helps to understand clinical trial details, expressive pharmacological action, agreements and collaborations, and the latest news and press releases.

Idiopathic Pulmonary Fibrosis Emerging Drugs

• Admilparant: Bristol-Myers Squibb

Admilparant (BMS-986278) is a potential first-in-class, oral, small molecule lysophosphatidic acid receptor 1 (LPA1) antagonist currently being evaluated as a novel antifibrotic treatment for patients with idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. Increased LPA levels and activation of LPA1 are involved in the pathogenesis of pulmonary fibrosis. A preclinical in vitro and in vivo study found that antagonizing LPA1 may be beneficial in treating lung injury and fibrosis. Currently, the drug is in the Phase III stage of its development for the treatment of Idiopathic Pulmonary Fibrosis.

• Taladegib (ENV-101): Endeavor BioMedicines

Taladegib (ENV-101) is an investigational, orally available small-molecule Hedgehog (Hh) signaling pathway inhibitor developed by Endeavor BioMedicines. The drug selectively inhibits Smoothened (SMO), a key transmembrane component of the Hh pathway, thereby blocking downstream Hh signaling involved in fibroblast-to-myofibroblast activation and fibrotic tissue remodeling. Currently, the drug is in the Phase II stage of its development for the treatment of Idiopathic Pulmonary Fibrosis.

• AZD8965: AstraZeneca

AZD8965 is an investigational oral small-molecule arginase inhibitor, arginase is involved in arginine metabolism and contributes to the production of ornithine, which can be further converted into proline, an important building block for collagen synthesis. By selectively inhibiting arginase, AZD8965 is designed to reduce ornithine availability and consequently limit collagen production and extracellular matrix deposition, thereby potentially slowing fibrotic remodeling in the lungs. Preclinical studies demonstrated reductions in arginase activity and collagen synthesis, supporting its development as an antifibrotic therapy for IPF. AstraZeneca is currently evaluating AZD8965 in the Phase IIb ARGiNAUT study in patients with Idiopathic Pulmonary Fibrosis.

• SRN001: siRNAgen Therapeutics Inc.

SRN-001 is a novel small interfering RNA (siRNA) drug being developed to treat fibrosis using Self Assembled Micelle inhibitory ribonucleic acid (SAMiRNA(TM)) technology. Amphiregulin (AREG) is a growth factor involved in fibroblast proliferation and myofibroblast transformation which is the hallmark of fibrosis in lung and kidney tissues. AREG is a downstream gene overexpressed by Transforming growth factor-B (TGF-B) during fibrosis, promoting fibroblast to myofibroblast transition (FMT). SRN-001 is designed to downregulate generating amphiregulin by RNA interference (RNAi). Currently, the drug is in the Phase I stage of its development for the treatment of Idiopathic Pulmonary Fibrosis.

• Cymerus(TM) MSCs: Cynata therapeutics

Cymerus(TM) MSCs are investigational mesenchymal stem cells (MSCs) derived from induced pluripotent stem cells (iPSCs) and developed by Cynata Therapeutics using its proprietary Cymerus(TM) platform. The technology generates MSCs through an intermediate cell type called mesenchymoangioblasts (MCAs), enabling the production of consistent, scalable, and allogeneic, off-the-shelf cell therapies from a single iPSC donor source. Currently, the drug candidate is in the Preclinical stage of its development for the treatment of Idiopathic Pulmonary Fibrosis.

Idiopathic Pulmonary Fibrosis: Therapeutic Assessment

This segment of the report provides insights about the different Idiopathic Pulmonary Fibrosis drugs segregated based on following parameters that define the scope of the report, such as:

• Major Players in Idiopathic Pulmonary Fibrosis

There are approx. 100+ key companies which are developing the therapies Idiopathic Pulmonary Fibrosis. The companies which have their Idiopathic Pulmonary Fibrosis drug candidates in the most advanced stage, i.e. Phase III include, Bristol-Myers Squibb and others.

• Phases

DelveInsight's report covers around 100+ products under different phases of clinical development like

• Late stage products (Phase III)
• Mid-stage products (Phase II)
• Early-stage product (Phase I) along with the details of
• Pre-clinical and Discovery stage candidates
• Discontinued & Inactive candidates
• Route of Administration

Idiopathic Pulmonary Fibrosis pipeline report provides the therapeutic assessment of the pipeline drugs by the Route of Administration. Products have been categorized under various ROAs such as

• Intra-articular
• Intraocular
• Intrathecal
• Intravenous
• Ophthalmic
• Oral
• Parenteral
• Subcutaneous
• Topical
• Transdermal
• Molecule Type

Products have been categorized under various Molecule types such as

• Oligonucleotide
• Peptide
• Small molecule
• Product Type

Drugs have been categorized under various product types like Mono, Combination and Mono/Combination.

Idiopathic Pulmonary Fibrosis: Pipeline Development Activities

The report provides insights into different therapeutic candidates in phase II, I, preclinical and discovery stage. It also analyses Idiopathic Pulmonary Fibrosis therapeutic drugs key players involved in developing key drugs.

Pipeline Development Activities

The report covers the detailed information of collaborations, acquisition and merger, licensing along with a thorough therapeutic assessment of emerging Idiopathic Pulmonary Fibrosis drugs.

Idiopathic Pulmonary Fibrosis Report Insights

• Idiopathic Pulmonary Fibrosis Pipeline Analysis
• Therapeutic Assessment
• Unmet Needs
• Impact of Drugs

Idiopathic Pulmonary Fibrosis Report Assessment

• Pipeline Product Profiles
• Therapeutic Assessment
• Pipeline Assessment
• Inactive drugs assessment
• Unmet Needs

Key Questions:

Current Treatment Scenario and Emerging Therapies:

• How many companies are developing Idiopathic Pulmonary Fibrosis drugs?
• How many Idiopathic Pulmonary Fibrosis drugs are developed by each company?
• How many emerging drugs are in mid-stage, and late-stage of development for the treatment of Idiopathic Pulmonary Fibrosis?
• What are the key collaborations (Industry-Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the Idiopathic Pulmonary Fibrosis therapeutics?
• What are the recent trends, drug types and novel technologies developed to overcome the limitation of existing therapies?
• What are the clinical studies going on for Idiopathic Pulmonary Fibrosis and their status?
• What are the key designations that have been granted to the emerging drugs?

Key Players

• Bristol-Myers Squibb
• Endeavor BioMedicines
• AstraZeneca
• Cynata therapeutics
• siRNAgen Therapeutics Inc.
• Regend Therapeutics
• AbbVie
• Eyestem Research Pvt Ltd.
• Incyte Corporation
• Syndax Pharmaceuticals
• Epirium Bio
• Oxcia
• Innovotherapeutics
• ScarTec Therapeutics
• Avanti Biosciences
• Agomab Therapeutics
• Oorja Bio
• Vicore Pharma
• Rein Therapeutics
• Spark Biopharma

Key Products

• Admilparant
• Taladegib (ENV-101)
• AZD8965
• Cymerus(TM) MSCs
• SRN001
• REGEND007
• REGEND001 (Aeriotemcel)
• ABBV-142
• Aircyte-AEC
• Axatilimab
• Research programme: Idiopathic pulmonary fibrosis
• OXC-201
• INV-005
• SCAR-a5-IPF
• ABI-171
• AGMB-447
• ORJ-001
• Buloxibutid
• LTI-03
• SBP-401