pirfenidone / Generic mfg. - LARVOL DELTA

Successful and practical pirfenidone desensitization. (PubMed, Tuberk Toraks) - No abstract available

Journal

Potential Therapeutic Strategies for Steatosis, Oxidative Stress, Inflammation, and Fibrosis in Liver Disease. (PubMed, Int J Mol Sci) - "Owing to its antioxidant and anti-inflammatory actions, NAC has been successfully used to treat chronic liver injuries, including hepatosteatosis and fibrosis. Therefore, ursodeoxycholic acid, pirfenidone, S-adenosyl-L-methionine, and N-acetylcysteine could represent therapeutic strategies for the treatment of liver pathologies."

Journal • Review • Cardiovascular • Fibrosis • Heart Failure • Hepatocellular Cancer • Hepatology • Idiopathic Pulmonary Fibrosis • Immunology • Infectious Disease • Inflammation • Influenza • Liver Cirrhosis • Liver Failure • Metabolic Disorders • Metabolic Dysfunction-Associated Steatohepatitis • Oncology • Polycystic Ovary Syndrome • Primary Biliary Cholangitis • Pulmonary Disease • Renal Disease • Respiratory Diseases • Solid Tumor • NLRC5

Combined Therapy with Pirfenidone, Metformin, and Mesenchymal Stem Cells Attenuates Bleomycin-Induced Pulmonary Fibrosis in Rats. (PubMed, Biomedicines) - " The combination of PFD, MET, and BM-MSCs offered superior therapeutic efficacy in treating BLM-induced pulmonary fibrosis compared to individual treatments. This multimodal approach effectively targets oxidative stress, inflammation, apoptosis, and fibrosis, suggesting strong potential for future clinical application."

IO biomarker • Journal • Preclinical • B Cell Lymphoma • Fibrosis • Immunology • Lymphoma • Oncology • Pulmonary Disease • Respiratory Diseases • BAX • COL1A1 • IL10 • MMP9 • TGFB1 • TNFA

Targeting the epithelium in pulmonary fibrosis. (PubMed, Eur Respir Rev) - "Consequently, drug discovery has focused on the activity of scar-forming fibroblasts, with some success shown by the pharmaceutical agents nintedanib and pirfenidone, and more recently nerandomilast. Here, we review the advances made in "omic" technologies, the approaching spatial profiling revolution, current and future functional cell systems, novel in vivo models, how we assign disease-relevance to test systems via computational gene set enrichment analysis, and link all these elements to clinically relevant biomarkers. We show how epithelial populations, identified and validated through cutting-edge technologies, may provide targets for the next wave of therapeutics for patients with IPF."

Journal • Review • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

DEVELOPMENT OF A HUMANIZED 3D BIOPRINTED MODEL FOR STUDYING KIDNEY FIBROSIS AND DRUG RESPONSE (ISN-WCN 2026) - "Fibrosis was induced using transforming growth factor-β1 (TGF-β1), and the anti-fibrotic effects of pirfenidone (PFD), a drug approved for pulmonary fibrosis, were evaluated...However, no significant impact on epithelial-mesenchymal transition or myofibroblast differentiation was observed via α-SMA staining.Conclusion This humanized 3D bioprinted model offers a physiologically relevant platform for studying kidney fibrosis and evaluating anti-fibrotic therapies. It overcomes limitations of conventional models, enabling mechanistic insights and high-throughput drug screening for future CKD treatment strategies."

Chronic Kidney Disease • Fibrosis • Immunology • Nephrology • Pulmonary Disease • Renal Disease • Respiratory Diseases • ACTA2 • COL1A1 • COL3A1 • COL4A1 • LOX • SMAD1 • TGFB1

Improved quality of life, reduced quantitative lung fibrosis in a trial of inhaled pirfenidone for idiopathic pulmonary fibrosis. (PubMed, BMC Pulm Med) - No abstract available

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

The effect of Nintedanib on KL-6 levels in patients with interstitial lung disease: A systematic review and meta-analysis. (PubMed, Autoimmun Rev) - "Anti-fibrotic therapy appears to stabilize KL-6 in ILD, supporting its prognostic utility. Baseline lung function may influence the KL-6 response. Larger, standardized prospective studies are needed to clarify KL-6's role as a dynamic biomarker of treatment response in ILD."

Journal • Retrospective data • Review • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases • Rheumatology

The mechanism of Rap1GAP in ventricular hypertrophy and interstitial fibrosis induced by angiotensin Ⅱ (PubMed, Zhonghua Xin Xue Guan Bing Za Zhi) - "For inhibitor rescue experiments, cardiac fibroblasts infected with Ad-Rap1GAP or Ad-GFP were treated with 10 μmol/L p38 inhibitor (SB203580) or TGF-β1 inhibitor (pirfenidone), forming Ad-Rap1GAP+AngⅡ+SB203580 group, Ad-Rap1GAP+Ang+pirfenidone group, Ad-GFP+AngⅡ+SB203580 group and Ad-GFP+Ang+pirfenidone group... Rap1GAP may mediate cardiomyocyte hypertrophy by regulating the mitogen-activated protein kinase signaling pathway and the expression of its downstream target NF-κB. Meanwhile, Rap1GAP may promote myocardial fibrosis by inducing ROS production and activating the TGF-β1/mitogen-activated protein kinase signaling pathway."

Journal • Fibrosis • Immunology • MAPK8 • PCNA • TGFB1

Double Immunosuppression With or Without Anti-fibrotic in Scleroderma ILD (clinicaltrials.gov) - P=N/A | N=35 | Recruiting | Sponsor: University of Patras

New trial • Immunology • Scleroderma • Systemic Sclerosis

Pirfenidone Induced Photosensitive Rash in Idiopathic Pulmonary Fibrosis: A Case Report (EAACI 2026) - No abstract available

Case report • Clinical • Immunology

A unified biochemical-physical regulatory nanoparticle modulates tumor mechanics to augment nanomedicine penetration and antitumor efficacy. (PubMed, J Control Release) - "Upon specific uptake by CAFs, elevated intracellular reactive oxygen species (ROS) trigger cleavage of the thioketal linker in PFD-TK-Cy, releasing modified pirfenidone (PFD-OH) to suppress pro-fibrotic signaling and a cyanine-based photosensitizer (SO3Cy) to generate localized mild hyperthermia (ca. This spatiotemporally coordinated strategy disrupts the vicious loop between CAFs and ECM, leading to reduced solid stress, improved blood perfusion, and enhanced intratumoral delivery of Doxil in stroma-rich orthotopic breast cancers. This study offers a promising nanoplatform for overcoming mechanical barriers in solid tumors."

Journal • Breast Cancer • Oncology • Solid Tumor • CAFs

Bexotegrast for treatment of idiopathic pulmonary fibrosis (BEACON-IPF): study protocol for a multinational, phase 2b/3, double-blind, randomised, multicentre, controlled trial. (PubMed, BMJ Open Respir Res) - P2 | "Background therapy with pirfenidone or nintedanib is permitted in ≤70% of the study population. Study results will be disseminated in peer-reviewed journals and international conferences targeted to medical, academic and patient communities. NCT06097260."

Journal • P2/3 data • P2b data • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Safety and Efficacy of Nerandomilast in Patients With Pulmonary Fibrosis: A Systematic Review and Meta-Analysis of Randomized Controlled Trials. (PubMed, Clin Respir J) - "Nerandomilast appears to slow lung function decline in pulmonary fibrosis without added safety risks. Although a lower pooled risk of mortality was observed, individual trials were not powered for mortality outcomes, and event rates were low; therefore, this finding should be interpreted cautiously. Given the heterogeneity of pulmonary fibrosis phenotypes and trial designs, further large-scale RCTs should explore standardized outcomes, subgroup effects, and combination strategies with nintedanib or pirfenidone."

Clinical • Journal • Retrospective data • Review • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

ANTIFIBROTICS IN RHEUMATOID ARTHRITIS-INTERSTITIAL LUNG DISEASE: COMPARATIVE STUDY OF NINTEDANIB VS PIRFENIDONE. NATIONAL MULTICENTER STUDY OF 107 PATIENTS (EULAR 2026) - No abstract available

Clinical • Immunology • Inflammatory Arthritis • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases • Rheumatoid Arthritis • Rheumatology

A Phase 1, Open-Label Study to Evaluate Pharmacokinetics and Drug-drug Interactions of ENV-101 (Taladegib) in Healthy Participants (clinicaltrials.gov) - P1 | N=57 | Not yet recruiting | Sponsor: Endeavor Biomedicines, Inc.

New P1 trial • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

CAR T cell therapy for fighting IPF: perspectives on a living drug. (PubMed, Front Immunol) - "Although two pharmacological agents (pirfenidone and nintedanib) have secured regulatory approval for clinical application, they remain incapable of substantially attenuating disease progression. These CAR T cells can specifically recognize and ablate fibroblasts expressing fibroblast activation protein (FAP). In this review, we summarize recently developed CAR T cell therapeutic strategies for IPF treatment with optimal targeting of FAP-fibroblasts, synthesize the existing preclinical studies and clinical trials evaluating anti-FAP CAR T cells to date, and critically discuss the adverse events associated with CAR T therapy alongside strategies to overcome current limitations of CAR T cell therapy in IPF management."

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

Design of a phase II randomised, double-blind, placebo-controlled, dose-finding trial of BI 1819479 in patients with idiopathic pulmonary fibrosis. (PubMed, ERJ Open Res) - P2 | "Patients with relevant airway obstruction (pre-bronchodilator forced expiratory volume in 1 s/FVC 15 mg·day-1 or equivalent, will be excluded. Treatment with approved IPF treatments (nintedanib/pirfenidone) is allowed if at a stable dose for ≥12 weeks prior to trial entry...Safety will be assessed throughout. This trial evaluates the efficacy, safety and dose range of BI 1819479 in patients with IPF, offering a potential additional treatment option, and will establish appropriate dosing for phase III trials."

Journal • P2 data • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Effects of the WNT signaling pathway on inflammation and fibrosis in idiopathic pulmonary fibrosis: Clinical, radiological and molecular evaluation. (PubMed, Exp Ther Med) - "Although antifibrotic agents such as pirfenidone and nintedanib can slow disease progression, these agents fail to reverse established structural damage, underscoring the urgent need for novel therapeutic strategies...The present study aimed to elucidate the role of WNT signaling in IPF pathogenesis, and to evaluate the antifibrotic and anti-inflammatory effects of the WNT inhibitors ETC-159 and LGK-974 in vitro...In conclusion, aberrant activation of WNT signaling may contribute to fibrogenesis and inflammation in IPF. Pharmacological inhibition of this pathway, particularly with LGK-974, exerts potent antifibrotic and anti-inflammatory effects, highlighting WNT signaling as a viable therapeutic target for IPF."

Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Inflammation • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases • IL1B • IL6 • TGFB1 • WNT7A

Industry Payments and Prescribing Patterns of Antifibrotic Therapy for Idiopathic Pulmonary Fibrosis in the United States. (PubMed, Respir Med) - "As of 2025, three antifibrotic drugs (pirfenidone, nintedanib, and nerandomilast) are approved for treatment. Receipt of drug-specific general payments was significantly associated with higher proportions of sponsored drug prescriptions for nintedanib (OR: 2.28; 95% CI: 2.15-2.41) and pirfenidone (OR: 1.94; 95% CI: 1.84-2.06). These findings highlight potential industry influence on clinical decision-making given clinical equipoise between antifibrotic agents."

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

Pirfenidone Inhibits Renal Fibrosis in Murine Lupus Nephritis via Downregulating MXRA7: A Potential Therapeutic Strategy (EULAR 2026) - No abstract available

Preclinical • Fibrosis • Glomerulonephritis • Immunology • Inflammatory Arthritis • Lupus • Lupus Nephritis • Nephrology • MXRA7

Detorsion is not innocent: pirfenidone prevents contralateral ovarian damage following ovarian torsion. (PubMed, Naunyn Schmiedebergs Arch Pharmacol) - "Post-detorsion pirfenidone therapy dose-dependently restored estrogen production, suppressed inflammasome activation (IL-1β, NLRP3, caspase-1) and fibrotic signaling (SMAD2, SMAD3, TGF-β1), and preserved normal follicular architecture in both ovaries. These findings demonstrate that the detorsed ovary remains a biologically active source of injury and identify pirfenidone as a potential adjunct therapy capable of protecting ovarian function after torsion."

Journal • Cardiovascular • Fibrosis • Gynecology • Reperfusion Injury • IL1B • NLRP3 • SMAD2 • SMAD3 • TGFB1

A case report of idiopathic pulmonary fibrosis concurrent with EGFR 19 deletion lung adenocarcinoma. (PubMed, Transl Cancer Res) - "The patient was managed with a combination therapy of icotinib (an EGFR-TKI) and Pirfenidone (an anti-fibrotic agent). Tumor molecular testing is also important in patients with NSCLC concurrent with IPF. Furthermore, it provides preliminary clinical evidence suggesting that, with careful monitoring and concomitant anti-fibrotic therapy, EGFR-TKIs like Icotinib may represent a viable treatment option for patients co-existing with IPF and EGFR-mutated NSCLC."

Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Infectious Disease • Interstitial Lung Disease • Lung Adenocarcinoma • Lung Cancer • Non Small Cell Lung Cancer • Novel Coronavirus Disease • Oncology • Pulmonary Disease • Respiratory Diseases • Solid Tumor • EGFR

Commercial Portfolio (GlobeNewswire) - "ETUARY (pirfenidone): Generated $106.1 million in sales of ETUARY for the full year ended December 31, 2025, compared to $105.0 million for the same period in 2024; Etorel (nintedanib ethanesulfonate soft capsules): Launched in June 2025 and generated $4.6 million in sales for the full year ended December 31, 2025; Contiva (avatrombopag maleate tablets): Launched in March 2025 and generated $5.5 million in sales for the full year ended December 31, 2025."

Generic launch • Sales • Idiopathic Pulmonary Fibrosis • Immune Thrombocytopenic Purpura • Interstitial Lung Disease

Targeting Inflammatory and Oxidative Pathways in Idiopathic Pulmonary Fibrosis: Synergistic Effects of Quercetin and Pirfenidone in Modulating Nrf2/PPAR-γ and TLR-4/NF-κB Pathways. (PubMed, Pharmacol Res Perspect) - "Our study was designed to explore the therapeutic potential of combining Pirf and Quer in mitigating bleomycin-induced lung fibrosis in vivo. Notably, the concomitant administration of Pirf and Quer always provided an augmented effect. The combined administration of Pirf and Quer significantly alleviated BLM-induced lung fibrosis more effectively than either drug alone, suggesting that this combination therapy could enhance anti-fibrotic treatments and should be further investigated in clinical settings."

Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Inflammation • Pulmonary Disease • Respiratory Diseases • IL1B • PI3K • PPARA • TGFB1 • TLR4 • TNFA

Methylophiopogonanone a attenuates pulmonary fibrosis by inhibiting SPP1-mediated macrophage polarization via the PI3K/Akt pathway. (PubMed, Animal Model Exp Med) - "This study identifies that MOA is a promising natural compound that alleviates pulmonary fibrosis by inhibiting SPP1-mediated macrophage polarization via the PI3K/Akt pathway."

Journal • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • IL4 • SPP1