Idiopathic Pulmonary Fibrosis Pipeline

DelveInsight’s, “Idiopathic pulmonary fibrosis - Pipeline Insight, 2025” report provides comprehensive insights about 80+ 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 progressive lung disease of unknown cause, marked by lung scarring and a poor long-term prognosis. It typically presents with gradually worsening shortness of breath, progressive dyspnea, and a dry, non-productive cough. Pulmonary function tests often show restrictive impairment, reduced forced vital capacity, and decreased carbon monoxide diffusing capacity. Early diagnosis is critical due to the rapid progression in advanced stages and can often be achieved without biopsy through careful evaluation of clinical history, imaging, and exclusion of other conditions.

The hallmark signs and symptoms of idiopathic pulmonary fibrosis (IPF) include progressive shortness of breath and a persistent dry cough, often accompanied by loss of appetite and gradual weight loss. Some patients may develop finger or toe clubbing due to chronic oxygen deficiency. These features are not specific to IPF, as other respiratory conditions can present with similar symptoms.

Idiopathic pulmonary fibrosis (IPF) is primarily driven by alveolar epithelial injury, often triggered by environmental factors such as smoking, chronic aspiration, infections, and advancing age, which activate fibroblasts and disrupt normal repair processes. This dysregulated repair leads to excessive extracellular matrix deposition, scarring, and destruction of lung architecture, impairing gas exchange and progressing to hypoxic respiratory failure. Smoking is a major risk factor, with 70–80% of patients affected, and around 30% exhibit concurrent emphysema, characterized by preserved lung volumes but reduced diffusing capacity (DLCO). IPF is commonly associated with comorbidities, including pulmonary hypertension, obstructive sleep apnea, coronary artery disease, venous thromboembolism, lung cancer, gastroesophageal reflux disease, hiatal hernia, osteoporosis, hypothyroidism, anxiety, and depression, all of which influence prognosis and patient management. The interplay of these factors underscores the complex pathophysiology of IPF, linking epithelial injury, aberrant fibroblast activity, and systemic comorbidities to progressive lung fibrosis and reduced survival.

International guidelines recommend that IPF diagnosis be made through a multidisciplinary approach, requiring exclusion of known causes of interstitial lung disease (ILD) and identification of a usual interstitial pneumonia (UIP) pattern on high-resolution CT (HRCT) or, when necessary, surgical lung biopsy. A “definite UIP” pattern, characterized by honeycombing with sub pleural, basal-predominant reticulation, with or without traction bronchiectasis, can be diagnosed on HRCT alone, eliminating the need for biopsy. Previously, a “possible UIP” pattern, lacking honeycombing but showing other features, required histological confirmation; however, surgical biopsies are often avoided in elderly or comorbid patients due to procedural risks. Evidence indicates that “possible UIP” on HRCT, in the appropriate clinical context, strongly predicts UIP at biopsy, allowing a working diagnosis of IPF. Despite these advances, IPF diagnosis remains complex and stressful, prompting exploration of innovations such as cryobiopsy and computer-assisted CT analysis to improve accuracy and consistency.

The pharmacological management of idiopathic pulmonary fibrosis (IPF) has evolved significantly, with current guidelines recommending disease-modifying therapies over anti-inflammatory or immune-targeting drugs. In contrast, two antifibrotic agents, pirfenidone and nintedanib, have emerged as disease-modifying therapies. Pirfenidone, an oral pyridine, exhibits anti-inflammatory, antioxidant, and antifibrotic effects by regulating TGF-β expression and inhibiting fibroblast and collagen synthesis, although its precise mechanism remains unclear. Nintedanib, a multi-tyrosine kinase inhibitor targeting PDGF, VEGF, and FGF receptors, has demonstrated prevention of lung fibrosis in preclinical models and reduced functional decline in phase 2 and 3 trials at 150 mg twice daily, earning approval for patients with mild-to-moderate IPF.

 

"Idiopathic pulmonary fibrosis- Pipeline Insight, 2025" 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.

Drug	Company	Phase	MoA	RoA
DUB program: USP30 Inhibitor	Mission Therapeutics	Preclinical	USP30 Inhibitor	NA
LTI-03	Lung Therapeutics	Phase I	Caveolin-1 replacements	Inhalation
TD-1058	Theravance Biopharma	Phase I	Transforming growth factor-beta type I receptor antagonists	Inhalation
PLN-74809	Pliant Therapeutics	Phase II	Integrin alphavbeta1 inhibitors; Integrin alphaVbeta6 inhibitors	Oral
GB0139	Galecto Biotech	Phase II	Galectin 3 inhibitors	Inhalation
Pamrevlumab	FibroGen	Phase III	Connective tissue growth factor inhibitors	Intravenous
PRM-151	Roche	Phase III	Protein replacements; Transforming growth factor beta1 modulators	Intravenous
C21	Vicore Pharma	Phase II	Angiotensin type 2 receptor agonists	Oral

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 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.

 

Idiopathic pulmonary fibrosis Emerging Drugs

• BMS-986278 : Bristol –Myers Squibb

BMS-986278 is an investigational, first-in-class oral small-molecule antagonist of lysophosphatidic acid receptor 1 (LPA1), being developed as a novel antifibrotic therapy for patients with idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF). Elevated lysophosphatidic acid (LPA) levels and subsequent activation of LPA1 play a critical role in the pathogenesis of pulmonary fibrosis, making it a promising therapeutic target. Recognizing its potential, the U.S. FDA has granted BMS-986278 both Fast Track and Orphan Drug designations for the treatment of IPF. Currently, the drug is in Phase III stage of its clinical study for the treatment of Idiopathic pulmonary fibrosis.

 

• Vixarelimab : Genetech Research

Vixarelimab (RG6536) is a fully human monoclonal antibody, administered subcutaneously,   designed to selectively target the OSMRβ receptor, thereby inhibiting both oncostatin M (OSM) and interleukin-31 (IL-31) signaling, while preserving signaling through the LIFR pathway. Dysregulation of OSM and OSMRβ has been implicated in a range of human diseases, where it may contribute to fibrosis, inflammation, and other pathological processes within affected tissues. The program is being developed under Genentech Research and Early Development, with rights in-licensed from Kiniksa Pharmaceuticals. Currently, the drug is in Phase II stage of its clinical study for the treatment of Idiopathic pulmonary fibrosis.

 

• TTI-101 : Tvardi Yherapeutics

TTI-101 is an oral small-molecule inhibitor of STAT3 that blocks its phosphorylation at tyrosine 705 by binding to the SH2 domain, thereby preventing dimerization and nuclear translocation. This selective mechanism inhibits STAT3’s canonical nuclear activity while preserving its essential mitochondrial functions. The U.S. FDA has granted TTI-101 orphan drug designation for idiopathic pulmonary fibrosis (IPF). Currently, the drug is in Phase II stage of its clinical study for the treatment of Idiopathic pulmonary fibrosis.

 

• ME-015 : Melius Pharma AB

ME-015 is an oral therapy with over 25 years of real-world safety data, making it well-suited for frail idiopathic pulmonary fibrosis (IPF) patients as it requires no monitoring or frequent physician visits. With no drug–drug interactions, it is particularly beneficial for IPF patients experiencing cough who are often on multiple medications. By reducing cough, ME-015 has the potential to significantly improve quality of life in patients who continue to face limited life expectancy despite existing treatment options. Currently, the drug is in Phase II stage of its clinical study for the treatment of Idiopathic pulmonary fibrosis.

 

• Saracatinab : AstraZeneca

Saracatinib is an oral, small-molecule inhibitor of src tyrosine kinase, originally discovered by AstraZeneca and previously studied in oncology. Leveraging a novel computational approach that repurposed drugs developed for other diseases, researchers from AstraZeneca, National Jewish Health, Mount Sinai Icahn School of Medicine, and Yale School of Medicine identified its potential in idiopathic pulmonary fibrosis (IPF). Recognizing this promise, the U.S. FDA has granted Saracatinib Orphan Drug Designation for IPF. Currently, the drug is in Phase I/II stage of its clinical study for the treatment of Idiopathic pulmonary fibrosis.

 

• HRS-9813: Guangdong Hengrui Pharmaceutical Co., Ltd

HRS-9813, a Class 1 innovative oral therapy developed by Hengrui Medicine, works by blocking fibrocyte recruitment to lung injury sites, thereby preventing their differentiation into myofibroblasts and slowing pulmonary fibrosis progression. Its sustained-release capsules have been approved for clinical trials in idiopathic pulmonary fibrosis (IPF). Currently, the drug is in Phase I stage of its clinical study for the treatment of Idiopathic pulmonary fibrosis.

 

• RSN0402: Shenzhen Resproly Biopharmaceutical Co., Ltd

RSN0402, developed by Resproly after three years of research, is an inhaled powder formulation designed as a precise lung-targeted therapy for idiopathic pulmonary fibrosis (IPF). By delivering the drug directly to the lungs, RSN0402 significantly reduces the required dose and minimizes gastrointestinal side effects commonly associated with oral treatments, while aiming to improve clinical efficacy. Preclinical studies have shown it to be safe, well tolerated, and effective in reducing lung fibrosis and inflammation. The initiation of its Phase I clinical trial, marked by the first patient enrollment, represents a key milestone for Resproly and a promising step toward advancing inhaled therapies for IPF. Currently, the drug is in Phase I stage of its clinical study for the treatment of Idiopathic pulmonary fibrosis.

Further product details are provided in the report……..

 

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. 80+ key companies which are developing the therapies for 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.

 

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

• 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.

 

Idiopathic pulmonary fibrosis: Pipeline Development Activities

The report provides insights into different therapeutic candidates in Phase III, 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?

Also Read @ Latest DelveInsight Blogs