pirfenidone / Generic mfg. - LARVOL DELTA

A Study of the Pharmacokinetic Interaction Between Pirfenidone, Nintedanib, and Nalbuphine Extended Release (NAL ER) in Healthy Participants (clinicaltrials.gov) - P1 | N=115 | Not yet recruiting | Sponsor: Trevi Therapeutics

New P1 trial

Strengthening the Assessment of Antifibrotic Therapy in Compensated Cirrhosis. (PubMed, Liver Int) - No abstract available

Journal • Fibrosis • Immunology

Pirfenidone, a drug intended to treat idiopathic pulmonary fibrosis, prevented CKD progression in an experimental model of hypertensive nephrosclerosis (ERA 2025) - "These animals were divided among the following groups: NAME (animals receiving only L-NAME with no therapeutic treatment, N=12), LOS: NAME (rats treated with 50mg/kg/day of Losartan, n=12) and PIRF: (NAME rats treated with 750mg/kg/day of pirfenidone, N=12). Our preliminary data indicate that PIRF promoted significant renoprotective effect in the NAME model of CKD, comparable to the protection achieved with LOS treatment. Such positive effect can be probably attributed to the antifibrotic action of PIRF, which may have contributed to reduce both the glomerular and the tubulointerstitial damage in NAME rats. Although further studies are still required in order to confirm our findings, here we suggest that pirfenidone may represent a promising therapeutic alternative in the treatment of progressive CKD."

Chronic Kidney Disease • Fibrosis • Glomerulonephritis • Idiopathic Pulmonary Fibrosis • Immunology • Inflammation • Nephrology • Pulmonary Disease • Respiratory Diseases • MMP2 • MMP9

Repurposing of the small-molecule adrenoreceptor-inhibitor carvedilol for treatment of the fibrotic lung. (PubMed, Front Pharmacol) - "Current therapies are very limited, with nintedanib and pirfenidone being the only non-invasive but non-curative interventions, ultimately bridging to lung transplantation. Herein, carvedilol demonstrated significant anti-fibrotic effects on human lung fibroblasts in vitro, thus presenting great potential as an anti-IPF treatment. In addition, miR-21 was validated as a secreted pro-fibrotic biomarker in the ex vivo PCLS model."

Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • Transplantation • MIR21

Sequential drug release system: Targeting the tumor ECM for enhanced chemotherapy efficacy. (PubMed, Proc Natl Acad Sci U S A) - "Upon administration, the hydrogel first releases pirfenidone to inhibit collagen production, weakening the ECM, followed by the release of paclitaxel, which improves tumor penetration. Single administration of the hydrogel led to long-term localized drug release, maintaining higher concentrations of chemotherapeutic agents in the tumor tissue and effectively reducing the tumor volume. This study provided a promising strategy to enhance chemotherapy in ECM-dense tumors, offering an efficient and minimally invasive method for localized, sustained-release cancer therapy."

Journal • Oncology • Oral Cancer • Pancreatic Cancer • Solid Tumor • Squamous Cell Carcinoma

The updated evidence of pirfenidone treated silicosis based on network pharmacology, molecular docking and experimental validation. (PubMed, Front Med (Lausanne)) - "In conclusion, we identified TNF, MMP9, NF-κB1 and TLR2, that contribute to the therapeutic effects of PFD in silicosis. Mechanistically, PFD appears to mitigate silicosis pathogenesis through suppression of epithelial TLR2/NF-κB pathway activation."

Journal • Fibrosis • Immunology • Inflammation • Pulmonary Disease • Respiratory Diseases • FN1 • MMP9 • TLR2

Role of pirfenidone in the primary prophylaxis of lung cancer in idiopathic pulmonary fibrosis patients: A systematic review and meta-analysis. (ASCO 2025) - "This systematic review and meta-analysis highlight the potential of pirfenidone as a primary prophylactic agent for lung cancer, demonstrating significant reductions in lung cancer incidence and risk. While findings are promising, further prospective studies are warranted to confirm these results, elucidate mechanisms, and establish optimal dosing regimens."

Retrospective data • Review • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Infectious Disease • Lung Cancer • Oncology • Pulmonary Disease • Respiratory Diseases • Solid Tumor

INTERSTITIAL LUNG DISEASE IN PRIMARY SJÖGREN SYNDROME: PATHOCHRONY, SERONEGATIVE CASES, AND RISK OF PROGRESSIVE PULMONARY FIBROSIS (EULAR 2025) - "Treatments included glucocorticoids in 77% of patients (33/43; mean initial dose of prednisone: 32 ± 14 mg/day), immunosuppressants in 77% (33/43; mycophenolate in 31 and azathioprine in 2), and biologic agents in 28% (12/43; rituximab in 11 and abatacept in 1). Antifibrotic agents (nintedanib or pirfenidone) were added in 26% (10/43) of patients due to progression to progressive pulmonary fibrosis (PPF)... In 78% of our cases, ILD was either the presenting symptom or the predominant manifestation that led to the diagnosis of pSS. This condition should be considered in patients with ILD and sicca syndrome, even in the absence of characteristic antibodies, as 38% of our cases were seronegative. At least 26% of patients progressed to PPF, requiring antifibrotic treatment."

Clinical • Fibrosis • Immunology • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Disease • Respiratory Diseases • Rheumatology • Sjogren's Syndrome

COMPARISON OF THE EXTENSION OF DIFFERENT TOMOGRAPHIC PATTERNS OF CONNECTIVE TISSUE DISEASE-ASSOCIATED INTERSTITIAL LUNG DISEASE AMONG DIFFERENT ANTIBODY SPECIFICITIES USING ARTIFICIAL INTELLIGENCE APPLICATION: A SINGLE-CENTER PILOT STUDY. (EULAR 2025) - "None of the patients had previous antifibrotic therapy with nintedanib or pirfenidone. The study showed significant differences in the VE of CTD-ILD tomographic patterns based on AAb profiles. In particular, it confirms, even through the use of software equipped with artificial intelligence, that the most severe ILD patterns are associated with positivity for anti-Scl-70 (for all tomographic patterns, such as BcE, GG, and HC) and the presence of M-AAb+ (for GG and HC patterns). It also shows that anti-Scl-70 is more associated with the greater presence of fibrosing pattern, as well as the presence of M-AAb+, and that ACPA positivity is associated with a higher probability of greater GG extension."

Clinical • Bronchiectasis • Fibrosis • Immunology • Infectious Disease • Inflammatory Arthritis • Interstitial Lung Disease • Myositis • Novel Coronavirus Disease • Oncology • Pulmonary Disease • Respiratory Diseases • Rheumatoid Arthritis • Rheumatology • Scleroderma • Sjogren's Syndrome • Systemic Sclerosis

Clinical outcomes of a multidisciplinary approach in patients with interstitial lung disease in anti-neutrophil cytoplasm antibody associated vasculitides: a real-world monocentric prospective study (EULAR 2025) - "At baseline, 19 patients were being treated with immunosuppressants (eight with rituximab (RTX), five with methotrexate, three with cyclophosphamide, one with azathioprine, hydroxychloroquine or mycophenolate mofetil); all were taking oral steroids (OCS) (9.4 ± 6.5 mg/die, expressed as prednisone equivalent). Two patients, both with MPA, were taking antifibrotics (one with nintedanib and one with pirfenidone) because of a previous diagnosis of idiopathic pulmonary fibrosis (IPF)... Our study suggests a different phenotype of AAV-ILD between MPA and GPA patients in accordance with available literature. In particular, MPA-ILD showed a greater similarity with IPF in terms of age and radiological pattern, as also suggested by the previous prescription of antifibrotic treatment, confirming the challenge of differential diagnosis. Our data suggests that a prompt evaluation and early treatment protocol shared between rheumatologist and pulmonologist is associated with a..."

Clinical • Clinical data • Real-world • Real-world evidence • ANCA Vasculitis • Idiopathic Pulmonary Fibrosis • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Disease • Rare Diseases • Respiratory Diseases • Rheumatology • Vasculitis • CRP

Role of antifibrotics in progressive pulmonary fibrosis associated to autoimmune diseases: NEREA Registry (EULAR 2025) - "This study presents a novel prospective longitudinal real-world comparison of antifibrotics in progressive pulmonary fibrosis-interstitial lung disease (PPF-ILD) of autoimmune origin. The rate of worsening was 57.4 per 100 patients-year. Both nintedanib and pirfenidone reduced the risk of worsening regardless of other factors."

Cardiovascular • Fibrosis • Immunology • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Disease • Respiratory Diseases • Rheumatology

Inhaled Pirfenidone as an innovative therapeutic approach to treat autoimmune ILD and other forms of Progressive Pulmonary Fibrosis: Phase 2b Study Design (EULAR 2025) - "Patients will remain on stable background immunosuppression and up to 30% of patients will remain on background nintedanib therapy. MIST is the first study to analyze the novel concept of inhaled medications in autoimmune ILD. Safety and efficacy of AP01 in patients with PPF will be studied. In addition to the safety and efficacy endpoints, MIST will examine the presence of cough in this population of patients."

P2b data • Cough • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Prescription FINO2 and Pirfenidone Supported in Reducing Fibrosis in Mouse Breast Tumor Tissue by Targeting SLC7A11 and HMOX1. (PubMed, Curr Top Med Chem) - "These findings suggest that FINO2 ferroptosis agonists, when combined with other anticancer agents like Pirfenidone, can enhance ferroptosis and reduce tumor fibrosis. Additionally, the overexpression of SLC7A11 and HMOX1 in breast cancer model mice is associated with increased tumor growth and reduced metastasis, indicating that targeting these proteins with specific inhibitors may be a promising strategy for breast cancer treatment."

Journal • Preclinical • Breast Cancer • Fibrosis • Immunology • Oncology • Solid Tumor • CD34 • HMOX1 • SLC7A11

Post-COVID pulmonary sequelae: Mechanisms and potential targets to reduce persistent fibrosis. (PubMed, Pharmacol Ther) - "Besides the approved anti-fibrotics, pirfenidone and nintedanib, other promising treatments include histone deacetylase inhibitors, angiotensin receptor blockers and mesenchymal stem cells. The pathophysiological mechanisms underlying post-COVID-19 pulmonary fibrosis are still incompletely understood, necessitating future research to clarify the development of persistent post-COVID-19 pulmonary fibrosis following SARS-CoV-2 infection. Given the widespread transmission of SARS-CoV-2, even a low prevalence of persistent post-COVID-19 pulmonary fibrosis would represent a significant public health concern for which therapeutic strategies are essential to identify."

Journal • Review • Fibrosis • Immunology • Infectious Disease • Novel Coronavirus Disease • Pulmonary Disease • Respiratory Diseases

Teton Phase 3 Clinical Trials of Inhaled Treprostinil in the Treatment of Idiopathic Pulmonary Fibrosis: Annual Update of Preliminary Baseline Data (ATS 2025) - P3 | "Stable background use of pirfenidone or nintedanib is allowed... The prospectively designed TETON Phase 3 clinical trials aim to definitively explore the hypothesis around inhaled treprostinil in IPF and may offer a much-needed inhaled treatment option for this vulnerable patient population."

Clinical • P3 data • Cardiovascular • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

TETON-PPF Clinical Trial of Inhaled Treprostinil for the Treatment of Progressive Pulmonary Fibrosis: Preliminary Baseline Demographics (ATS 2025) - P3 | "Background use of pirfenidone or nintedanib is allowed... The prospectively designed TETON-PPF Phase 3 clinical trial is being performed in parallel with the TETON studies for IPF to avoid any delay in this potential treatment option becoming available for PPF patients, who currently have limited treatment options but a similar prognosis as IPF patients."

Clinical • Cardiovascular • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Real-world insights into safety, tolerability, and predictive factors of adverse drug reactions in treating idiopathic pulmonary fibrosis with pirfenidone and nintedanib. (PubMed, Ther Adv Drug Saf) - "Identifying ADR predictors is essential for personalizing treatment strategies. Both pirfenidone and nintedanib are crucial in managing IPF, highlighting the need for further research to optimize personalized therapies and patient outcomes."

Adverse drug reaction • Biomarker • Journal • Real-world evidence • Anorexia • Cardiovascular • Hepatology • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • Transplantation

3,4-Dimethoxycinnamic acid from coffee silverskin biowaste ameliorates bleomycin-induced pulmonary fibrosis via modulating caveolin-1-dependent activation of NF-κB, TGF-β1/Smad3, and ERK1/2 signaling pathways. (PubMed, Toxicol Appl Pharmacol) - "In conclusion, DMCA effectively mitigates bleomycin-induced lung fibrosis in rats, which is comparable to pirfenidone, through antioxidant, anti-inflammatory, and anti-fibrotic effects. These beneficial effects are mainly mediated through boosting caveolin-1 expression that halts NF-κB, TGF-β1/smad3, and ERK1/2 signaling pathways."

Journal • Fibrosis • Immunology • Oncology • Pulmonary Disease • Respiratory Diseases • CAV1 • IL1B • SMAD3 • TGFB1 • TNFA

Biodegradable Double-Layer Hydrogels with Sequential Drug Release for Multi-Phase Collaborative Regulation in Scar-Free Wound Healing. (PubMed, J Funct Biomater) - "The lower layer, containing curcumin-loaded chitosan nanoparticles, shows early anti-inflammatory and antioxidant effects, while the upper layer, with pirfenidone-encapsulated gelatin microspheres, presents late-stage anti-fibrotic activity...In a rat model of full-thickness skin defect, treatment with a double-layer hydrogel drug delivery system accelerated the wound closure, improved scar quality, and promoted the formation of hair follicles. Therefore, this innovative approach lays a promising foundation for future clinical applications in anti-scar therapies, offering a significant advancement in wound care and regenerative medicine."

Journal • Inflammation

Initiation of Supplemental Oxygen in the FIBRONEER-IPF Trial of Nerandomilast in Patients With Idiopathic Pulmonary Fibrosis (ATS 2025) - " Patients with IPF were randomized 1:1:1 to receive nerandomilast 9 mg bid, nerandomilast 18 mg bid, or placebo, stratified by background antifibrotic therapy (nintedanib/pirfenidone vs none). In the FIBRONEER-IPF trial in patients with IPF, a reduced risk of initiation of supplemental oxygen during follow-up was observed in patients receiving nerandomilast 9mg bid versus placebo."

Clinical • Late-breaking abstract • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Epidemiology of Combined Pulmonary Fibrosis and Emphysema (CPFE) Among a National Cohort of United States Veterans (ATS 2025) - "Antifibrotic (nintedanib or pirfenidone) utilization was higher in patients with IPF alone (3.7% vs 1.5%, p<.01). CPFE is highly prevalent among Veterans and is associated with greater comorbidity burden, worse pulmonary function, and higher mortality rates. How these patients interface with the healthcare system and the care they receive deserves further investigation."

Clinical • Late-breaking abstract • Cardiovascular • Congestive Heart Failure • Heart Failure • Idiopathic Pulmonary Fibrosis • Immunology • Lung Cancer • Obstructive Sleep Apnea • Oncology • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases • Sleep Apnea • Sleep Disorder • Solid Tumor

Efficacy and Safety of Pirfenidone in Patients With Progressive Pulmonary Fibrosis: A Retrospective Single-center Study in South Korea (ATS 2025) - "Of the two antifibrotics used as standard treatment for IPF, international guidelines recommended the use of nintedanib for PPF, while evidence supporting pirfenidone remains insufficient. The use of pirfenidone in patients with PPF has the potential to reduce the rate of FVC decline in real clinical practice. The adverse events of pirfenidone in patients with PPF were similar to those previously reported in IPF and were mostly mild."

Late-breaking abstract • Retrospective data • Anorexia • Idiopathic Pulmonary Fibrosis • Immunology • Inflammation • Inflammatory Arthritis • Interstitial Lung Disease • Pneumonia • Pruritus • Pulmonary Disease • Respiratory Diseases • Rheumatoid Arthritis • Rheumatology • Sjogren's Syndrome

Real-World Treatment Persistence and Predictive Factors for Discontinuation of Antifibrotic Therapies in Patients With Idiopathic Pulmonary Fibrosis: A Post-hoc Analysis of Two Multicenter Observational Cohort Studies in Poland (ATS 2025) - "In this large population-based cohort of patients with IPF, the rate of discontinuation of antifibrotic therapies was 34.4% over the study follow-up. Additionally, the rates and time to discontinuation of treatment were not different between subjects starting pirfenidone or nintedanib. Clinical predictive factors including age, BMI, TLco% predicted, GAP index score, and use of LTOT were associated with the risk of discontinuation of antifibrotic medications."

Biomarker • Clinical • HEOR • Late-breaking abstract • Observational data • Real-world • Real-world evidence • Retrospective data • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Single Cell Analysis of Peripheral Blood Reveals Immune Cell-specific Expression Profiles Predictive of Clinical Outcomes in Fibrotic Hypersensitivity Pneumonitis (ATS 2025) - P2 | " Single-cell RNA sequencing in peripheral blood mononuclear cells (PBMCs) was obtained from 40 patients with fibrotic HP (fHP) enrolled in the prospective pirfenidone randomized clinical trial (NCT02958917)... Peripheral blood single-cell transcriptomics may aid in patient stratification and inform clinical decisions while identifying key molecular targets for fHP progression."

Clinical • Clinical data • Immune cell • Fibrosis • Immunology • Inflammation • Pneumonia • Pulmonary Disease • CD14 • CD8

CFTR Modulator VX770 Inhibits Cell Senescence and Mitigates Bleomycin Induced Pulmonary Fibrosis in Mouse Lung (ATS 2025) - "To date, only Pirfenidone and Nintedanib have been approved by the FDA for the treatment for IPF patients. Our results demonstrate that VX770 mitigates the BLM-induced pulmonary fibrosis in mice in part by inhibiting inflammation and AT2 cell senescence. There are controversies about whether VX770 potentiates murine CFTR, thus the mechanism of action in these studies remains to be determined. This study thus provides evidence of the anti-fibrotic role of VX770 and warrants for further investigations of its therapeutic potential in treatment of IPF."

Preclinical • Cystic Fibrosis • Fibrosis • Genetic Disorders • Idiopathic Pulmonary Fibrosis • Immunology • Inflammation • Pulmonary Disease • Respiratory Diseases • CCL2 • CDKN1A • COL1A1 • CTHRC1 • IL6 • KRT7 • RUNX1 • TGFB1 • VIM