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Progressive pulmonary fibrosis (PPF) is a chronic lung condition marked by relentless scarring that leads to impaired breathing and declining lung function. According to Rare Disease Advisor, idiopathic pulmonary fibrosis, a key PPF subtype, shows global incidence around 10 cases per 100,000 people annually, with considerable regional variation. The progressive pulmonary fibrosis pipeline analysis by Expert Market Research highlights rising prevalence and expanding therapeutic focus on antifibrotic agents, immunomodulators, and novel mechanisms targeting fibrosis progression. There is growing focus on precision medicine, regenerative treatments, and advanced diagnostics to slow disease progression. With unmet needs and active clinical trials, the PPF drug pipeline and market are poised for significant growth in the coming years.
Major companies involved in the progressive pulmonary fibrosis pipeline analysis include Bristol-Myers Squibb, Avalyn Pharma Inc., and others.
Leading drugs currently in the pipeline include BMS-986278, AP01, HEC585, and others.
The pipeline shows strong growth driven by increasing late-stage candidates, expanded mechanism diversity, and rising biologic therapies targeting fibrotic pathways, inflammation control, and slowing disease progression.
The Progressive Pulmonary Fibrosis Pipeline Analysis Report by Expert Market Research gives comprehensive insights into progressive pulmonary fibrosis therapeutics currently undergoing clinical trials. It covers various aspects related to the details of each of these drugs under development for progressive pulmonary fibrosis. The progressive pulmonary fibrosis report assessment includes the analysis of over 100 pipeline drugs and 50+ companies. The progressive pulmonary fibrosis pipeline landscape will include an analysis based on efficacy and safety measure outcomes published for the trials, including their adverse effects on patients suffering from the condition, and alignment with progressive pulmonary fibrosis treatment guidelines to ensure optimal care practices.
The assessment part will include a detailed analysis of each drug, drug class, clinical studies, phase type, drug type, route of administration, and ongoing product development activities related to progressive pulmonary fibrosis.

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Progressive pulmonary fibrosis is a chronic lung disorder characterized by continuous scarring of lung tissue, leading to a gradual decline in respiratory function. It develops as a complication of various interstitial lung diseases, including autoimmune conditions and environmental exposures. Persistent inflammation triggers fibrosis, reducing oxygen transfer and causing worsening breathlessness over time.
Progressive pulmonary fibrosis treatment is done through antifibrotic therapies, immunomodulators, supportive oxygen therapy, pulmonary rehabilitation, and treatment of underlying interstitial lung diseases to slow lung function decline and improve quality of life. In December 2025, Boehringer Ingelheim received U.S. Food and Drug Administration approval for JASCAYD (nerandomilast), a preferential phosphodiesterase 4B inhibitor, for progressive pulmonary fibrosis. The drug demonstrated significant slowing of lung function decline in Phase III clinical trials, marking a major advancement in the disease treatment pipeline.
The global incidence of idiopathic pulmonary fibrosis (IPF) is estimated at approximately 10 cases per 100,000 people annually, though rates vary across regions, according to Rare Disease Advisor. In the United States, a 2011 study of Medicare beneficiaries aged 65 years and older reported an incidence of 93.7 cases per 100,000 people and a prevalence of 494.5 cases per 100,000 people. As per Huajian Liu et al., 2024, real-world data from the United Kingdom indicate a higher prevalence of progressive pulmonary fibrosis than previously estimated, with up to 20% of patients starting nintedanib presenting with advanced disease. According to Maria S. Nashatyreva et al., 2023, the overall proportion of IPF was 13.7% in India and 11.6% in the greater Paris region. These findings highlight the growing recognition and burden of progressive pulmonary fibrosis globally.
This section of the report covers the analysis of progressive pulmonary fibrosis drug candidates based on several segmentations, including:
By Phase
The pipeline assessment report covers 50+ drug analyses based on phase:
By Drug Class
The progressive pulmonary fibrosis pipeline analysis report covers 50+ drug analyses based on drug classes:
By Route of Administration
The pipeline assessment report covers 50+ drug analyses based on the route of administration:
The report covers phase I, phase II, phase III, phase IV, and early-phase drugs. The coverage includes an in-depth analysis of each drug across these phases. According to EMR analysis, phase II, with 47%, covers a major share of the total progressive pulmonary fibrosis clinical trials, highlighting strong midstage activity and promising therapeutic candidates. Phase III accounts for 33%, indicating significant late‑stage progression and potential near‑term market approvals. Phase I contributes 13%, reflecting early clinical exploration, while phase IV holds 7%, demonstrating post‑marketing evaluation. Together, these phases underscore a balanced and advancing pipeline that can drive innovation, expand treatment options, and positively impact market growth.
The drug molecule categories covered under the progressive pulmonary fibrosis pipeline analysis include small molecules, monoclonal antibodies, recombinant proteins, and cell therapies. The progressive pulmonary fibrosis report provides a comparative analysis of the drug classes for each drug in various phases of clinical trials for progressive pulmonary fibrosis. In the pipeline, phosphodiesterase 4B (PDE4B) inhibitors are emerging as promising therapeutic options. For instance, nerandomilast, an oral preferential PDE4B inhibitor, is under investigation in Phase III trials for idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. It works by reducing inflammation and slowing lung function decline, demonstrating a favorable safety and tolerability profile compared to placebo.
The EMR report for the progressive pulmonary fibrosis pipeline covers the profile of key companies involved in clinical trials and their drugs under development. It provides a detailed progressive pulmonary fibrosis therapeutic assessment, analyzing the competitive dynamics of the clinical trial landscape. Below is the list of a few players involved in progressive pulmonary fibrosis clinical trials:
This section covers the detailed analysis of each drug under multiple phases, including phase I, phase II, phase III, phase IV, and emerging drugs for progressive pulmonary fibrosis. It includes product description, trial ID, study type, drug class, mode of administration, and recruitment status of progressive pulmonary fibrosis drug candidates.
BMS-986278 is an oral, small-molecule lysophosphatidic acid receptor 1 (LPA1) antagonist being developed by Bristol-Myers Squibb as a potential first-in-class antifibrotic therapy for progressive pulmonary fibrosis. The ongoing Phase 3, multicenter, randomized, double-blind, placebo-controlled study is evaluating the efficacy, safety, and tolerability of the drug in affected patients. The study is examining whether blocking LPA1 signaling can reduce fibrosis progression by inhibiting pathways involved in lung injury and tissue scarring, aiming to slow disease advancement and improve clinical outcomes.
AP01 (Inhaled Pirfenidone) is being developed by Avalyn Pharma Inc. as a targeted antifibrotic therapy for progressive pulmonary fibrosis. The ongoing Phase 2b randomized, double-blind, placebo-controlled study is evaluating the safety and efficacy of two inhaled doses over 52 weeks. The objective is to examine lung function stabilization, measured primarily by forced vital capacity, while improving tolerability. AP01 is a novel inhalation formulation designed to deliver pirfenidone directly to the lungs, reducing systemic exposure and enhancing localized antifibrotic activity.
HEC585 (Yinfenidone) is an oral antifibrotic small-molecule drug derived from pirfenidone, developed with enhanced metabolic stability, a longer half-life, and increased biological activity to improve treatment outcomes in fibrotic lung diseases. The drug is working by inhibiting fibroblast activation and reducing collagen deposition through modulation of TGF-β–mediated signaling pathways that drive pulmonary fibrosis progression. Sponsored by Sunshine Lake Pharma Co., Ltd., this Phase IIb randomized, double-blind, placebo-controlled study is evaluating two oral doses of HEC585 in patients with progressive pulmonary fibrosis. The objective of this ongoing study is to examine the drug’s efficacy in delaying Forced Vital Capacity decline while continuously assessing its safety and long-term tolerability over extended treatment periods.
*Please note that this is only a partial list; the complete list of drugs will be available in the full report.*
The Progressive Pulmonary Fibrosis Pipeline Analysis Report provides a strategic overview of the latest and future landscape of treatments for progressive pulmonary fibrosis. It provides necessary information for making informed investment decisions along with research, development, and strategic planning efforts. The stakeholders will benefit from the essential insights into progressive pulmonary fibrosis collaborations, regulatory environments, and potential growth opportunities.
*While we strive to always give you current and accurate information, the numbers depicted on the website are indicative and may differ from the actual numbers in the main report. At Expert Market Research, we aim to bring you the latest insights and trends in the market. Using our analyses and forecasts, stakeholders can understand the market dynamics, navigate challenges, and capitalize on opportunities to make data-driven strategic decisions.*
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