pirfenidone / Generic mfg. - LARVOL DELTA

First-line treatment with low-dose ruxolitinib, pirfenidone, and low-dose calcineurin inhibitors leads to significant improvement in patients with bronchiolitis obliterans syndrome after allogeneic transplantation (ASH 2025) - "Traditional treatments for BOS rely on glucocorticoids combined with FAM regimens ( fluticasone + azithromycin + montelukast ) and calmodulin phosphatase inhibitors ( CNIs ), but they have limited efficacy with an even lower overall remission rate ( ORR ) and poor prognosis once progression to steroid-refractory cGVHD (SR-cGVHD) is achieved...Methods A retrospective analysis of 14 patients, 11 males and 3 females, with a median age of 19 years (7-54 years), who underwent hematopoietic stem cell transplantation combined with BOS at Henan Cancer Hospital from 2018 to 2025, including 6 cases of mild BOS, 3 cases of moderate BOS, 5 cases of severe BOS, Among them, 6 cases of mild, 3 cases of moderate, and 5 cases of severe BOS were treated with low-dose rucotinib ( 5 mg qd po) combined with piroxicam (200 mg tid po), small-dose CNI (half of the effective therapeutic concentration), and FAM first-line treatment...Conclusions In conclusion, the first-line treatment of BOS..."

Clinical • Bone Marrow Transplantation • Chronic Graft versus Host Disease • Graft versus Host Disease • Infectious Disease • Pulmonary Disease • Respiratory Diseases • Transplantation

The single-center retrospective analysis of steroid-free rmap regimen (ruxolitinib, montelukast, azithromycin, and pirfenidone) treating moderate-to-severe bronchiolitis obliterans syndrome (BOS). (ASH 2025) - "The steroid-free RMAP regimen demonstrated promising efficacy in moderate-to-severe BOS,enabling rapid steroid withdrawal while improving quality of life and long-term survival. However, giventhe small sample size and retrospective design, further prospective studies are warranted for validation."

Retrospective data • Bone Marrow Transplantation • Infectious Disease • Pneumonia • Pulmonary Disease • Respiratory Diseases

L-arginine-induced chronic pancreatitis in mice: Evaluating effects of pirfenidone and simvastatin. (PubMed, World J Gastrointest Pharmacol Ther) - "Combination of pirfenidone and simvastatin demonstrated a synergistic therapeutic effect in reducing inflammation, fibrosis, and oxidative stress in an L-arginine-induced chronic pancreatitis mouse model, suggesting promise for chronic pancreatitis management."

Journal • Preclinical • Fibrosis • Immunology • Oncology • Pancreatitis • GPX1 • IL10 • TGFB1 • TNFA

Efficacy of pirfenidone in fibrotic hypersensitivity pneumonitis: a systematic review and meta-analysis of randomized controlled trials. (PubMed, Ann Med Surg (Lond)) - "However, there was a significant decrease in the SGRQ score at the end of the intervention [MD: -5; 95% CI: -6.88, -3.12; P < 0.00001]. Pirfenidone was not associated with a significant change in the lung function metrics, but it has been shown to improve the quality of life, as evidenced by a significant decrease in the SGRQ score."

Journal • Retrospective data • Fibrosis • Immunology • Inflammation • Interstitial Lung Disease • Pneumonia • Pulmonary Disease • Respiratory Diseases

A Single-arm Clinical Study Evaluating Pirfenidone and Sintilimab in Combination With Standard Neoadjuvant Chemotherapy for Colorectal Cancer Patients With Peritoneal Metastasis. (clinicaltrials.gov) - P2 | N=30 | Not yet recruiting | Sponsor: Guoxiang Cai

New P2 trial • Colorectal Cancer • Oncology • Solid Tumor

In vitro assessment of pirfenidone anti-fibrotic properties in a humanized 3D bioprinted kidney tubulointerstitium model. (PubMed, Biomed Pharmacother) - "However, no clear effect on the epithelial-mesenchymal transition (EMT) and cellular differentiation into myofibroblasts was shown with immunostaining for alpha-smooth muscle actin (α-SMA). In conclusion, we demonstrated that the developed bioprinted model could be used to test drugs for kidney fibrosis, offering an alternative to current models with more pathophysiological relevant conditions."

Journal • Preclinical • Chronic Kidney Disease • Fibrosis • Immunology • Nephrology • Pulmonary Disease • Renal Disease • Respiratory Diseases • TGFB1

Biotechnology-based therapies for mitigation of pulmonary fibrosis: an update. (PubMed, Drug Discov Today) - "The treatment for PF is currently limited to two drugs: nintedanib and pirfenidone. This review provides key insights on biotechnology-based advancements for PF, namely monoclonal antibodies, peptides, nucleic acids and stem cell therapy. We also underscore the obstacles and prospective developments in biotechnology-derived therapeutics for PF."

Journal • Review • Fibrosis • Immunology • Inflammation • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Hydronidone Mitigates Pulmonary Fibrosis by Regulating the TGF-β/Smad2/3 Axis in in Vitro and in Vivo Models. (PubMed, ACS Pharmacol Transl Sci) - "Although antifibrotic drugs like nintedanib and pirfenidone can slow disease progression, their clinical benefits remain modest...In an in vivo manner, a bleomycin (BLM)-induced PF mouse model was employed...Hydronidone effectively reduces pulmonary fibrotic marker expression and improves lung function at lower doses (25 and 50 mg/kg) than pirfenidone (100 mg/kg) without compromising the safety profile. These findings support its potential as a promising antifibrotic agent and warrant further clinical investigation."

Journal • Preclinical • Fibrosis • Immunology • Liver Cirrhosis • Pulmonary Disease • Respiratory Diseases • SMAD2 • SMAD4 • TGFB1

Artesunate attenuates pulmonary fibrosis by suppressing fibroblast senescence through inhibition of the STAT3/p53 signaling pathway. (PubMed, Toxicol Appl Pharmacol) - "Art demonstrates therapeutic potential for IPF by inhibiting fibroblast senescence through STAT3/p53 signaling axis suppression."

Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • CDKN1A • TGFB1

Structure-Based Optimization of Imidazopyridine Derivatives as Selective and Orally Bioavailable Phosphodiesterase 10A Inhibitors with Reduced Blood-Brain Barrier Penetration for the Treatment of Idiopathic Pulmonary Fibrosis. (PubMed, J Med Chem) - "In a bleomycin-induced murine model of PF, oral administration of QC-3 (10 mg/kg, once daily) demonstrated superior antifibrotic efficacy compared to pirfenidone (300 mg/kg, once daily), while exhibiting minimal cerebral residue, thereby reducing its potential risk of central nervous system suppression. Moreover, QC-3 attenuates PF by blocking myofibroblast differentiation through the cAMP/PKA/CREB signaling pathway, highlighting that inhibition of PDE10A provides a novel and promising therapeutic strategy for PF."

Journal • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Post-COVID Interstitial Lung Disease: Pirfenidone Might Play a Significant Role in Resolution (APSR 2025) - "She was diagnosed with post-COVID ILD (PC-ILD) and started on intravenous methylprednisolone pulsed therapy followed by oral steroids. This highlights the need for early recognition, appropriate management, and long-term follow-up in PC-ILD. Pirfenidone may play a pivotal role in fibrotic types of PC-ILD."

Asthma • Bronchiectasis • Cough • Fibrosis • Immunology • Infectious Disease • Interstitial Lung Disease • Novel Coronavirus Disease • Pneumonia • Pulmonary Disease • Respiratory Diseases

Impact of virtual nurse/pharmacist-led monitoring in patients with interstitial lung disease (ILD) on antifibrotic treatment in a UK ILD specialist centre (BTS WM 2025) - "Background Antifibrotic medications are prescribed for Idiopathic Pulmonary Fibrosis (Pirfenidone or Nintedanib) and Progressive Fibrosing ILD (Nintedanib) with the aim of slowing disease progression. Conclusion Our preliminary ILD data demonstrates an annual increase in length of antifibrotic treatment since 2020, which could be related to the initiation of virtual RN/RPh-led monitoring. A comparative analysis of pre-initiation data would provide further insights into the role of RN/RPh in improving antifibrotic tolerance, as well as explore other variables that may influence sustained therapy maintenance."

Clinical • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Antifibrotic prescribing in a tier 2 centre: the ILD integrated clinical care pathway in practice (BTS WM 2025) - "Introduction Nintedanib and pirfenidone reduce the rate of progression in fibrotic lung diseases. ILD Care Pathway [online]. Available from: https://cdn.prod.website-files.com/5e32c31d3ab26b41eba332b2/661fc1e24752a5ebfbbfd567_OneVoiceILD%20Care%20Pathway%20report.pdf"

Clinical • Fibrosis • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Real-world healthcare resource utilization in patients with idiopathic pulmonary fibrosis initiating antifibrotic therapy in the medicare fee-for-service population (BTS WM 2025) - "Methods A retrospective cohort study was conducted using the 100% Medicare Fee-For-Service data to identify patients who initiated antifibrotic therapy (nintendanib or pirfenidone) between 01/01/17 and 12/31/22...Conclusions Patients with IPF have high treatment discontinuation to antifibrotic therapy, hospitalizations and mortality. A large unmet need remains in patients with idiopathic pulmonary fibrosis."

Clinical • HEOR • Medicare • Real-world • Real-world evidence • Reimbursement • US reimbursement • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Real-world evaluation of patient characteristics impacting tolerability of antifibrotic treatment in interstitial lung diseases (BTS WM 2025) - "Background Pirfenidone and Nintedanib are antifibrotic medications licensed in the UK for Idopathetic Pulmonary Fibrosis (IPF), with Nintedanib also approved for Progressive Fibrosis Interstitial Lung Diseases (PFILD). Values represent averages, proportions or percentages corresponding to p-values to indicate significance Conclusion In this real-world cohort, antifibrotic therapy was tolerated by most patients, with better persistence observed in younger individuals and those on Nintedanib. Other baseline factors showed no clear association with tolerability."

Clinical • HEOR • Real-world • Real-world evidence • Cardiovascular • CNS Disorders • Coronary Artery Disease • Depression • Diabetes • Fibrosis • Gastroenterology • Gastrointestinal Disorder • Heart Failure • Hypertension • Immunology • Interstitial Lung Disease • Metabolic Disorders • Mood Disorders • Psychiatry • Pulmonary Disease • Respiratory Diseases

Impact of tiered service delivery model on antifibrotic adherence and mortality among patients with interstitial lung disease (BTS WM 2025) - "While there was no difference in discontinuation rate between tiers, pirfenidone had a significantly higher discontinuation risk compared to nintedanib (HR 1.60, 95% CI 1.05–2.44, p=0.029). View this table: View inline View popup Download powerpoint Abstract P25 Table 1 Overview of services offered by tier status in Merseyside ILD service Conclusions Our data suggest shared care tiered service delivery for AF delivery is safe, without difference in adherence or mortality between specialist and non-specialist sites. These findings support rollout of tiered service delivery within the UK."

Adherence • Clinical • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Defining the antifibrotic mechanisms of treprostinil in pulmonary fibrosis (BTS WM 2025) - "Fibroblast proliferation was also inhibited in a concentration-dependent manner by Treprostinil and there were additive effects when combined with nintedanib, pirfenidone, or nerandomilast. Antifibrotic effects of Treprostinil were detected in IPF derived PCLS. Further studies are ongoing to establish the underlying mechanism as there were additive effects of Treprostinil with established antifibrotic agents which may have important clinical implications."

Cardiovascular • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • RHOA • SERPINE1 • TGFB1

Baicalin inhibits the Akt/mTOR/ULK1 signaling pathway to activate autophagy and ameliorate pulmonary fibrosis. (PubMed, Int Immunopharmacol) - "Baicalin alleviated pulmonary fibrosis by activating autophagy and inhibiting EMT via the Akt/mTOR/ULK1 pathway. Our research offers new pharmacological insights into IPF treatment that targets autophagy."

Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Inflammation • Pulmonary Disease • Respiratory Diseases • TGFB1

Maintenance of Effectiveness of Pirfenidone in Elderly Patients with Progressive Functional Impairment: A Real-World Retrospective Study in IPF. (PubMed, Biomedicines) - "The intra-individual trend also appeared to be preserved when analyzing a small group of patients who had passed any two criteria. Neither attaining advanced age nor the development of severe functional impairment appeared to limit the effectiveness of pirfenidone."

Journal • Real-world evidence • Retrospective data • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Combining Pirfenidone With Mycophenolate Mofetil for Systemic Sclerosis-Related Interstitial Lung Disease (Scleroderma Lung Study III): An Investigator-Initiated, Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial. (PubMed, ACR Open Rheumatol) - "In this underpowered study, there was no statistically significant treatment difference in overall change in FVC-% between groups. MMF+PFD was not as well tolerated as MMF+PLA."

Clinical • Journal • P2 data • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis

Molecular mechanısms of ovarian fibrosis. (PubMed, Mol Hum Reprod) - "Current therapeutic strategies remain largely experimental, focusing on antifibrotic agents such as pirfenidone, TGF-β inhibitors and modulation of oxidative stress, alongside emerging interventions such as stem cell therapies, which are offer potential avenues for intervention in the ovary. This review synthesizes current insights into the cellular and molecular mechanisms driving ovarian fibrosis, its association with reproductive disorders, and emerging therapeutic strategies. It underscores key knowledge gaps and emphasizes the need for future research focused on fibroblast activation, inflammatory signaling and immune-ECM interactions to facilitate the development of targeted, long-term interventions aimed at preventing or reversing ovarian fibrosis and preserving female fertility."

Journal • Endometriosis • Fibrosis • Gynecology • Immunology • Infertility • Oncology • Ovarian Cancer • Polycystic Ovary Syndrome • Sexual Disorders • Solid Tumor • Women's Health • TGFB1

Sustained drug-releasing hydrogel coatings for ureteral stents to prevent iatrogenic injury-induced ureteral stricture. (PubMed, Mater Today Bio) - "Here, we explore the anti-stricture effects of commercial ureteral stents modified with hydrogel coatings, which contain functionalized poly (lactic-co-glycolic acid) (fPLGA) nanoparticles (NPs) loaded with the drug of pirfenidone (PFD)...More importantly, the slow degradation of fPLGA leads to sustained delivery of antifibrotic PFD, with a cumulative drug release rate of approximately 82 % within 12 weeks. These combined benefits contribute to the prevention of ureteral strictures after iatrogenic injury, as evidenced by inhibited fibrosis, alleviated oxidative stress, and suppressed inflammatory response in both vitro and in vivo studies."

Journal • Fibrosis • Immunology • Inflammation

New analogs of 5-substituted-2(1H)-pyridone containing of natural amino acids as potential drugs in idiopathic pulmonary fibrosis. Investigation in silico and preliminary in vitro. (PubMed, Acta Biochim Pol) - "Preliminary studies show that the designed compounds exhibit promising potential as anti-fibrotic therapeutics. Therefore, their activity is worth further exploring."

Journal • Preclinical • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • CXCR4 • IL17A • TGFB1 • TGFBR1

Efficacy and Safety of Pirfenidone Capsules in the Treatment of Pneumoconiosis (clinicaltrials.gov) - P3 | N=272 | Recruiting | Sponsor: Beijing Continent Pharmaceutical Co, Ltd. | Not yet recruiting ➔ Recruiting | Trial completion date: Sep 2025 ➔ Sep 2026 | Trial primary completion date: Sep 2024 ➔ Sep 2026

Enrollment open • Trial completion date • Trial primary completion date • Respiratory Diseases

The Effect of Antifibrotic Drugs in Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study Using Nationwide Data in Japan (APSR 2025) - "Patients were categorized into two groups: those who received antifibrotic drugs (nintedanib and pirfenidone) within 14 days of hospitalization and those who did not. The in-hospital mortality rate was 35% in the antifibrotic drug group and 37% in the non-antifibrotic drug group, with no statistically significant difference observed. Conclusion : The results of this study did not demonstrate a prognostic improvement effect of antifibrotic drugs after the onset of acute exacerbation of IPF."

Observational data • Retrospective data • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases