New drugs for pulmonary fibrosis Until 2014, lung transplantation was the only available treatment ― along with supportive therapies including oxygen, pulmonary rehabilitation, and the management of comorbidities such as obstructive sleep apnea or Jun 8, 2022 · Fibrosis, defined as the pathologic accumulation of extracellular matrix, is the final outcome of several common chronic inflammatory, immune-mediated, and metabolic diseases and accounts for up May 15, 2022 · Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible lung disease with high mortality. IPF is a disease caused by a build-up of scar tissue in the lungs called fibrosis, which causes the lungs to become stiffer and lose their elasticity The studies tested a new oral medication called nerandomilast and results show it slowed the decline in lung function over one year in people living with IPF and other forms of pulmonary fibrosis — both chronic lung diseases with limited treatment options that deeply affect daily life. Help may be on the way as a drug being tested locally aims at giving sufferers their breath back. May 19, 2025 · In a phase 3, double-blind trial, we randomly assigned patients with progressive pulmonary fibrosis in a 1:1:1 ratio to receive nerandomilast at a dose of 18 mg twice daily, nerandomilast at a Idiopathic pulmonary fibrosis (IPF) remains a fatal and incurable disease despite the use of approved antifibrotic drugs. Current drug development focuses on attenuating fibrotic responses via multiple pathways. New drug shows early promise in clinical trial for patients with IPF Professor Toby Maher An international clinical trial, led by Professor Toby Maher , which involved 17 centres in the UK, Italy and Ukraine, has shown some promising results for patients with idiopathic pulmonary fibrosis (IPF). Buloxibutid works by targeting the renin-angiotensin system, which plays a role in the progression of IPF. com provides accurate and independent information on more than 24,000 prescription drugs, over-the-counter medicines and natural products. Neither can reverse lung scarring or ease the disease’s debilitating symptoms, which, according to director of Yale Interstitial Lung Disease Center of Excellence Erica Herzog, include Sep 14, 2024 · Pulmonary fibrosis turns the lungs to scar tissue, making it harder and harder to breathe. The efficacy and safety of Apr 10, 2024 · For many progressive lung diseases like idiopathic pulmonary fibrosis (IPF), a key issue is a low supply of new stem cells to repair and reverse damage. Sep 12, 2018 · While multiple trials assessing different mechanisms and approaches to treatment have proved negative over the past decade and a half, two drug therapies have recently become available for the directed treatment of idiopathic pulmonary fibrosis (IPF). Jan 8, 2025 · Researchers at Vanderbilt University Medical Center and the University of Michigan Medical School have identified, in lab studies, a potential new treatment for idiopathic pulmonary fibrosis (IPF), a progressive, incurable lung disease that is on the rise in the United States. Subscribe to our newsletter for the latest medication news, new drug approvals and FDA alerts. New medicines and May 29, 2024 · 1. However, given that clinical practice guidelines have not addressed the application of this therapy, large well-designed and multicentre trials are warranted to provide a better picture of IPF management. Currently, two agents, pirfenidone and nintedanib are approved for this disease, and both have been shown to reduce the rate of decline in lung function in patients with IPF. Oct 25, 2023 · BMS-986278 (Bristol Myers Squibb) is a potential first-in-class treatment for progressive pulmonary fibrosis and idiopathic pulmonary fibrosis. 1,2 Currently, there are two approved antifibrotic drugs — nintedanib and pirfenidone Feb 10, 2025 · Nerandomilast, an oral phosphodiesterase 4B inhibitor, hit the primary endpoint in a Phase III trial in progressive pulmonary fibrosis—nearly six months after achieving a similar feat in idiopathic pulmonary fibrosis. [PMC free article] [Google Scholar] Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive-fibrosing lung disease with a median survival of less than 5 years. Dec 4, 2024 · Idiopathic pulmonary fibrosis: Vicore’s buloxibutid and BMS’ BMS-986278 chart wins . Since significant advances in the understanding of pathogenetic mechanisms in IPF, novel potential agents are being tested to identify new targeted and Medications for Pulmonary Fibrosis Other names: Asbestosis; Interstitial pneumonitis, from asbestos exposure; Pulmonary fibrosis, from asbestos exposure Idiopathic pulmonary fibrosis involves scarring or thickening of tissues deep in the lung without a known cause. (2022) Saracatinib, a Selective Src Kinase Inhibitor, Blocks Fibrotic Responses in Preclinical Models of Pulmonary Fibrosis. Prednisone, azathioprine, and N‐acetylcysteine for pulmonary fibrosis. For many of those with the most common form of the disease, idiopathic pulmonary fibrosis, life expectancy is less than five years. N Engl J Med. The promising 12-week data showed a reduction in the rate of lung function decline in patients with idiopathic pulmonary fibrosis (IPF). The study provides pharmacological proof of concept for an upcoming Phase The first results of this study show that the drug may help people with IPF. The pathogenesis is complex and encompasses multiple molecular pathways. The new drug has been developed by Boehringer Ingelheim (BI). Newton CA, Zhang D, Oldham JM, et al. [PMC free article] [Google Scholar] 39. Oct 27, 2023 · The U. BI is a family-owned biopharmaceutical company that developed the antifibrotic Nintedanib/Ofev, which is a current treatment option for some people living with pulmonary fibrosis (PF). Telomere length and use of immunosuppressive medications in idiopathic pulmonary fibrosis. Approved medication for pulmonary fibrosis. These findings are consistent with previously reported data from a group Nov 18, 2024 · A year ago, there was 1 drug for idiopathic pulmonary fibrosis in trials at Temple Health. May 29, 2024 · A promising drug for idiopathic pulmonary fibrosis (IPF) proved to be no better than a placebo at decreasing the rate of disease progression among participants in a multinational clinical trial. Oct 18, 2019 · Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with a median survival of 2. Experimental Pharmacologic Treatments for Idiopathic Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Disease Phase 3 Trials. IPF is rapidly progressive and causes scarring in the lungs, making it difficult to Mar 14, 2024 · Idiopathic pulmonary fibrosis (IPF) presents a clinical challenge characterized by progressive fibrosis and destruction of lung tissue. This systematic review evaluates the antifibrotic potential of non-antifibrotic agents and investigational drugs. Apr 26, 2024 · Researchers have developed an inhalable prototype medicine that may be able to repair lung scarring caused by idiopathic pulmonary fibrosis (IPF) by stimulating the growth of stem cells directly in the lungs, a study reports. It carries a median survival of two to three years, but clinical course Mar 1, 2023 · Drugs such as glucocorticoids, antioxidants, and receptor inhibitors are commonly used to treat PF in the early stage. The first-generation antifibrotics pirfenidone and nintedanib, approved more than 10 years ago, have been shown to reduce the rate of progression, increase the length of life for patients with IPF, and work for other fibrotic lung diseases. Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a progressive and devastating lung disease, characterized by progressive lung scarring. 5 to 5 years. Jun 29, 2022 · Similarly to idiopathic pulmonary fibrosis (IPF), other interstitial lung diseases can develop progressive pulmonary fibrosis (PPF) characterized by declining lung function, a poor response to immunomodulatory therapies, and early mortality. “Selectively targeting ROCK2 is an exciting, novel approach which could provide a new treatment option for patients with IPF, and with potential applications in other interstitial lung diseases and cancer-associated fibrosis,” Robertson said. American Journal of Respiratory and Critical Care Feb 10, 2025 · Boehringer’s Nerandomilast Meets Primary Endpoint in Phase III Study FIBRONEER™-ILD, in Progressive Pulmonary Fibrosis Ingelheim, Germany, February 10, 2025 - Boehringer Ingelheim announced today that the FIBRONEER™-ILD trial met its primary endpoint, which was the absolute change from baseline in forced vital capacity (FVC) [mL] at week Oct 30, 2024 · LA JOLLA, CA—The Calibr-Skaggs Institute for Innovative Medicines, the nonprofit drug development division of Scripps Research, announced today that the first dose of a pioneering regenerative lung therapy, CMR316, has been given in a phase 1 trial assessing safety and tolerability in healthy volunteers and patients with idiopathic pulmonary fibrosis (IPF). Aug 25, 2023 · In preclinical models, zelasudil showed the ability to lessen scarring — and it was generally well tolerated in a Phase 1 study of healthy volunteers. Sep 1, 2023 · Pulmonary fibrosis has a serious impact on the respiratory function of the human body, and the clinical manifestation is progressive dyspnea. Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, et al. , et al. 2012;366:1968‐1977. In the Apr 1, 2024 · New drugs and treatment strategies are urgently needed to improve the quality of life for patients with pulmonary fibrosis. In earlier trials, the drug, pemrevlumab, had appeared to slow disease progression. Disease progression in IPF is driven by a dysregulated cycle of microinjury, aberrant wound healing, and propagating fibrosis. Jan 15, 2025 · Idiopathic pulmonary fibrosis (IPF) is a severe, progressive fibrotic disease of the lung of unknown etiology that affects approximately 150,000 patients in the United States. Development of novel antifibrotics drugs has consistently increased over the last decades, but unsolved issues remain about endpoints, duration, and inclusion/exclusion criteria of future clinical trials. Aug 23, 2022 · An experimental anticancer drug called saracatinib shows promise as a treatment for Idiopathic Pulmonary Fibrosis (IPF), a chronic and often fatal condition that causes scarring or fibrosis of the lungs and makes breathing difficult. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT Clinical Practice Guideline. It is a rare disease of unknown origin that is manifested by alveolar inflammation, myofibroblast proliferation, and excessive extracellular matrix deposition, resulting in destruction of the normal lung architecture and impairment of lung function [Citation 1]. 1164/rccm. Phosphodiesterase 4 inhibitors (PDE4i), lysophosphatidic acid (LPA) antagonists, du … May 19, 2022 · Boehringer Ingelheim has announced Phase II data for BI 1015550, a novel investigational phosphodiesterase 4B (PDE4B) inhibitor. Nov 11, 2020 · A new treatment option for lung fibrosis is being developed by Purdue University scientists. Patients living with IPF experience disabling symptoms and progressive loss of lung function. Feb 15, 2024 · To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. Breakthrough therapy designation is intended to expedite the development and review of medicines for serious or life-threatening diseases. May 7, 2025 · Hear Bruce Ellsworth, PhD, scientific executive director, Medicinal Chemistry, and Aditya Patel, MD, clinical program lead for pulmonology, discuss how Bristol Myers Squibb is leveraging its deep expertise in drug discovery and disease biology to investigate potential treatments for pulmonary fibrosis. They found six key genes—ANO9, BRCA1, CCDC200, EZH1, FAM13A, and SFR1—as potential drug targets. 2 This compound is an investigational agent and has not been approved for use. Areas covered: Prior to antifibrotic therapy (pirfenidone and nintedanib), there was no validated pharmaceutical therapy for IPF. Log In to Reply Oct 26, 2022 · Ingelheim, Germany and Ridgefield, Conn, October 26, 2022 – Boehringer Ingelheim today announced that the first patient has enrolled in FIBRONEER™-IPF, a global Phase III trial evaluating BI 1015550, an investigational phosphodiesterase 4B (PDE4B) inhibitor, in people living with idiopathic pulmonary fibrosis (IPF). The pathophysiology of disordered lung repair involves common downstream pathways that lead to pulmonary fibrosis in both IPF and PPF. Drugs. Introduction. 1. These findings are consistent with previously reported data from a group Nov 11, 2020 · A new treatment option for lung fibrosis is being developed by Purdue University scientists. People with idiopathic pulmonary fibrosis (IPF Sep 25, 2023 · The experimental oral therapy BMS-986278, taken at 60 mg twice daily for 26 weeks, resulted in a 69% reduction in the rate of lung function decline in adults with progressive pulmonary fibrosis taking part in a Phase 2 trial. Nov 12, 2023 · Currently approved drug treatments for idiopathic pulmonary fibrosis (IPF), pirfenidone and nintedanib, have been shown to slow lung function decline and improve clinical outcomes. 202202-0399ST. 2022;205:e18–e47. Apr 22, 2025 · Researchers at Tulane University have identified a potential new way to treat idiopathic pulmonary fibrosis (IPF), a deadly and currently incurable lung disease that affects more than 3 million people worldwide. The antifibrotic Jun 7, 2024 · Medication for pulmonary fibrosis depends on the type of PF. Feb 10, 2023 · Currently, there are two FDA-approved drugs which treat pulmonary fibrosis, but these drugs act only to slow the rate of pulmonary fibrosis progression. People with idiopathic pulmonary fibrosis (IPF Apr 24, 2025 · Researchers at Tulane University have identified a potential new way to treat idiopathic pulmonary fibrosis (IPF), a deadly and currently incurable lung disease that affects more than 3 million people worldwide. Sep 16, 2024 · Nerandomilast (BI 1015550) is an investigational oral, preferential inhibitor of phosphodiesterase 4B (PDE4B) that is being studied as a potential treatment for idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF). BI 1015550 and PLN-74809 are experimental compounds that showed positive results in phase 2 studies, while Tyvaso is a approved therapy that may slow IPF progression. Lung fibrosis has been a concern for COVID-19 patients. Despite recent advancements, including antifibrotic medications like pirfenidone and nintedanib, IPF remains a chronic and often fatal condition with limited treatm … Sep 25, 2023 · The experimental oral therapy BMS-986278, taken at 60 mg twice daily for 26 weeks, resulted in a 69% reduction in the rate of lung function decline in adults with progressive pulmonary fibrosis taking part in a Phase 2 trial. Use the filters on the left-hand side to narrow results by study attributes. doi: 10. Food and Drug Administration (FDA) has granted breakthrough therapy designation to BMS-986278, Bristol Myers Squibb’s investigational therapy for progressive pulmonary fibrosis (PF). This year, Swedish biotech Vicore’s buloxibutid has also raised the stakes for a new idiopathic pulmonary fibrosis drug to enter the market. Boehringer plans to submit for FDA and other global approvals in both indications. IPF is rapidly progressive and causes scarring in the lungs, making it difficult to breathe. Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrosing interstitial lung disease that occurs predominantly in the older population. 24/7 Live Philadelphia Pennsylvania New Jersey Delaware Oct 17, 2024 · It’s a disease that can literally take your breath away—and there’s no cure. These cells are responsible for regenerating and increasing the growth of healthy tissue—without them, lung function decreases and a range of severe illnesses can take hold. Since significant advances in the understanding of pathogenetic mechanisms in IPF, novel potential agents are being tested to identify new targeted and better Jul 18, 2022 · Learn about the latest developments of novel drugs for idiopathic pulmonary fibrosis (IPF), a chronic lung disease. Aug 1, 2022 · Pathways targeted by pulmonary fibrosis drugs and drug candidates are primarily inhibitors of relevant targeting signaling pathways (the autotaxin and TGFβ pathways), as well as the pathway that . Oct 26, 2023 · Idiopathic pulmonary fibrosis (IPF) remains a disease with poor survival. For more information on the terminology used in drug development, please review the PF Pipeline Glossary. This NMA and cost-effectiveness analysis suggests that NAC + pirfenidone is the most cost-effective option for treatment of IPF at WTP thresholds of $150,000 and $200,000. One of the most common progressive forms of fibrosis of the lung is idiopathic pulmonary fibrosis (IPF). An international clinical trial led by Professor Toby Maher at Royal Brompton and Harefield hospitals (RBHH) has shown promising results for patients with idiopathic pulmonary fibrosis (IPF). As pulmonary fibrosis progresses, it causes scarring in the lungs, making it harder and harder to absorb oxygen into the bloodstream. There are several phase 3 studies underway that are studying the efficacy and safety of new medications for idiopathic pulmonary fibrosis (IPF) and progressive fibrosing ILD (PF-ILD). Idiopathic pulmonary fibrosis (IPF) is a debilitating and fatal condition that causes severe scarring of the lungs. Keywords: idiopathic pulmonary fibrosis (IPF), treatment, pharmacotherapy, anti-fibrotic, novel, adjunct, pathophysiology, etiology. Nintedanib is an anti-fibrotic drug that is approved in the United States to treat idiopathic pulmonary fibrosis, scleroderma-associated ILD (SSc-ILD), and chronic interstitial lung diseases in which fibrosis continues to progress. Am J Respir Crit Care Med . Pirfenidone, a well-known anti-fibrotic drug Pulmonary hypertension (PH) associated with interstitial lung disease (ILD) includes a diverse collection of up to 200 different pulmonary diseases, including interstitial pulmonary fibrosis, chronic hypersensitivity pneumonitis, connective tissue disease-related ILD, and chronic pulmonary fibrosis with emphysema (CPFE) among others. Idiopathic pulmonary fibrosis (IPF) remains a disease with poor survival. 1 The 2 treatments approved by the US Food and Drug Administration to treat IPF, nintedanib Mar 20, 2025 · To find new drug targets for Idiopathic Pulmonary Fibrosis (IPF), a team used a comprehensive multi-omics approach integrating genomic and transcriptomic data. These drugs reduce pulmonary fibrosis (PF) via acting on cytokines and receptors. 3 days ago · The new data from the 1,178 The two positive studies mean that Boehringer can now seek approval for nerandomilast with a broad label claim in pulmonary fibrosis, making the drug a potential Idiopathic pulmonary fibrosis (IPF) currently affects about 100 000 individuals in the US, and millions globally. Introduction: Currently approved drug treatments for idiopathic pulmonary fibrosis (IPF), pirfenidone and nintedanib, have been shown to slow lung function decline and improve clinical outcomes. there is a urgent need for new drug Welcome to the Pulmonary Fibrosis (PF) Drug Development Pipeline where you can learn about the latest in drug development for PF-related conditions. Now there are 10 active trials and 8 more coming up. S. Apr 22, 2025 · Researchers have identified a potential new way to treat idiopathic pulmonary fibrosis (IPF), a deadly and currently incurable lung disease that affects more than 3 million people worldwide. . The FDA has granted Breakthrough Therapy Designation to BMS-986278 (Bristol Myers Squibb), a potential first-in-class oral lysophosphatidic acid receptor 1 antagonist (LPA 1 ), for the treatment of Idiopathic pulmonary fibrosis clinical research network. Some types of medication include anti-fibrotic drugs for idiopathic pulmonary fibrosis, steroids, anti-acid medications and drugs to treat cough. The incidence of pulmonary fibrosis-related diseases is increasing year by year, and no curative drugs have appeared so far. The first-generation antifibrotics pirfenidone and nintedanib, approved more than 10 years ago, have been shown to reduce the rate of progression, increa … Oct 13, 2022 · Ahangari, F. wmj nqmwf acl wtrb vle slomt diebkd zca mwdrrfd bwhvq