Pulmonary Fibrosis - Pipeline Insight, 2025

This “Pulmonary Fibrosis - Pipeline Insight, 2025” report provides comprehensive insights about 110+ companies and 140+ pipeline drugs in 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.

Pulmonary Fibrosis: Understanding

Pulmonary Fibrosis: Overview

Pulmonary fibrosis is a chronic and progressive lung disease characterized by the excessive accumulation of extracellular matrix (ECM) and remodeling of the lung architecture. This leads to scarring and thickening of the lung tissue, which impairs gas exchange and results in a gradual decline in respiratory function. The disease is clinically recognizable by symptoms such as shortness of breath, chronic cough, and fatigue, with radiographic imaging revealing characteristic patterns of interstitial fibrosis.

The pathogenesis of pulmonary fibrosis (PF) involves complex interactions between genetic susceptibility, environmental triggers, and aberrant cellular responses. One of the key features of PF is the dysregulation of wound healing processes. Normally, when the lung is injured, the body activates repair mechanisms involving inflammation, epithelial cell regeneration, and collagen deposition. However, in pulmonary fibrosis, this repair process goes awry. Instead of resolving the injury and restoring normal tissue architecture, the wound healing process becomes chronic, leading to excessive fibrosis. This is believed to occur due to an imbalance in pro-fibrotic and anti-fibrotic signals within the lung. Specifically, the TGF-β (transforming growth factor-beta) signaling pathway, which is central to fibrosis development, is often dysregulated in PF, leading to sustained fibroblast activation, collagen production, and deposition in the lung interstitial space.

Pulmonary fibrosis (PF) presents with a variety of clinical symptoms, and the course of the disease can differ greatly from one patient to another. Although the early signs may be subtle, the disease generally progresses over time, leading to significant respiratory and systemic symptoms. One of the most common early symptoms of pulmonary fibrosis is shortness of breath. Initially, patients may only experience breathlessness during physical exertion, such as walking uphill or climbing stairs. However, as the disease advances, shortness of breath can occur with even mild activity or, in some cases, at rest. This progression occurs as the lungs become increasingly scarred, leading to a decreased capacity for gas exchange, thereby impairing the ability to absorb oxygen and expel carbon dioxide efficiently.

Non-pharmacological treatment strategies are essential in managing pulmonary fibrosis, focusing on symptom relief, improving physical function, and maintaining the patient's overall quality of life. In addition to general preventive measures, such as vaccination against pneumococcus and influenza to reduce the risk of respiratory infections, long-term oxygen therapy is a cornerstone of non-pharmacological treatment. Oxygen therapy is often used when necessary to improve oxygenation, and while it is typically recommended at rest, recent studies suggest that its use during exertion can also result in significant improvements in the patient's quality of life, although the effect size may be modest.

'Pulmonary Fibrosis- Pipeline Insight, 2025' report outlays comprehensive insights of present scenario and growth prospects across the indication. A detailed picture of the Pulmonary Fibrosis pipeline landscape is provided which includes the disease overview and Pulmonary Fibrosis treatment guidelines. The assessment part of the report embraces, in depth 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, 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 Pulmonary Fibrosis R&D. The therapies under development are focused on novel approaches to treat/improve Pulmonary Fibrosis.

Pulmonary Fibrosis Emerging Drugs Chapters

This segment of the Pulmonary Fibrosis report encloses its detailed analysis of various drugs in different stages of clinical development, including phase III, 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.

Pulmonary Fibrosis Emerging Drugs

BMS-986278: Bristol-Myers Squibb

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 LPA are involved in the pathogenesis of pulmonary fibrosis. BMS-986278 is a potent and complete antagonist of LPA action at LPA1-mediated Gi, Gq, G12, and β-arrestin signaling pathways in both cells heterologously expressing human LPA1 and in primary human lung fibroblasts. The drug is currently in Phase III stage of clinical trial evaluation for the treatment of pulmonary fibrosis.

AK3280: Ark Biosciences Inc.

AK3280 is a next-generation broad-spectrum anti-fibrotic molecule optimized from the marketed drug pirfenidone. It has the ability to modulate multiple pathways and biomarkers closely associated with the fibrotic process, including the expression of fibrosis-related genes and proteins induced by transforming growth factor-beta (TGF-B) and lysophosphatidic acid (LPA). AK3280 works by reducing cell proliferation and inhibiting the synthesis and accumulation of extracellular matrix. Compared to pirfenidone, AK3280 offers advantages in safety and tolerability, with potentially much better clinical efficacy. The drug is currently in Phase II stage of clinical trial evaluation for the treatment of pulmonary fibrosis.

LYT-100: PureTech Health

LYT-100 (deupirfenidone) is currently in development for idiopathic pulmonary fibrosis (IPF), which is a rare, progressive and fatal disease. LYT-100 is a deuterated form of pirfenidone and is designed to retain the beneficial pharmacology and clinically-validated efficacy of pirfenidone with a highly differentiated pharmacokinetic (PK) profile. In multiple clinical trials, LYT-100 has demonstrated a favorable tolerability profile, which may keep patients on treatment longer to enable more optimal disease management. The drug is currently in Phase II stage of clinical trial evaluation for the treatment of pulmonary fibrosis.

ARO-MMP7: Sarepta Therapeutics

ARO-MMP7 is an investigational RNA interference (RNAi) therapeutic developed by Arrowhead Pharmaceuticals, aimed at treating idiopathic pulmonary fibrosis (IPF) by targeting and reducing the expression of matrix metalloproteinase 7 (MMP7). This protein is implicated in the pathogenesis of IPF, contributing to inflammation and fibrosis in the lungs. The drug is currently in Phase I/II stage of clinical trial evaluation for the treatment of pulmonary fibrosis.

TRK-250: Toray Industries, Inc

TRK-250, also known as BNC-1021, is a nucleic acid medicine developed by Toray Industries in collaboration with BONAC Corporation. It is designed to treat Idiopathic Pulmonary Fibrosis (IPF). The drug works by selectively inhibiting the expression of transforming growth factor-beta 1 (TGF-β1), a key protein involved in the fibrotic process at the gene expression level. The drug is currently in Phase I stage of clinical trial evaluation for the treatment of pulmonary fibrosis.

VUM02: Wuhan Optics Valley Vcanbiopharma Co., Ltd.

VUM02 Injection is an innovative therapeutic product developed by Wuhan Optics Valley Vcanbiopharma Co., Ltd. It utilizes human umbilical cord-derived mesenchymal stem cells (hUCT-MSCs). The drug is currently in Phase I stage of clinical trial evaluation for the treatment of pulmonary fibrosis.

Pulmonary Fibrosis: Therapeutic Assessment

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

Major Players in Pulmonary Fibrosis

• There are approx. 110+ key companies which are developing the therapies for Pulmonary Fibrosis. The companies which have their Pulmonary Fibrosis drug candidates in the most advanced stage, i.e. Phase III include, Bristol-Myers Squibb.

Phases

The report covers around 140+ 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

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

• Oral
• Intravenous
• Subcutaneous
• Parenteral
• Topical

Molecule Type

Products have been categorized under various Molecule types such as

• Recombinant fusion proteins
• Small molecule
• Monoclonal antibody
• Peptide
• Polymer
• Gene therapy

Product Type

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

Pulmonary Fibrosis: Pipeline Development Activities

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

Pulmonary Fibrosis Report Insights

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

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 Pulmonary Fibrosis drugs?
• How many 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 Pulmonary Fibrosis?
• What are the key collaborations (Industry-Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the 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 Pulmonary Fibrosis and their status?
• What are the key designations that have been granted to the emerging drugs?

Key Players

• Bristol-Myers Squibb
• Ark Biosciences Inc.

PureTech Health

• Sarepta Therapeutics
• Toray Industries, Inc
• Wuhan Optics Valley Vcanbiopharma Co., Ltd.

Nitto Denko

• Syndax Pharmaceuticals
• Endeavor BioMedicines
• AstraZeneca
• Pulmongene Ltd.

BreStem Therapeutics

• Nuformix
• AbbVie
• Saniona

Key Products

• BMS-986278
• AK3280
• LYT-100
• ARO-MMP7
• TRK-250
• VUM02
• ND-L02-s0201
• Axatilimab
• ENV-101
• AZD5055
• PMG1015
• BRS 001
• NXP002
• ABT-263
• SAN-903

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