pirfenidone / Generic mfg. - LARVOL DELTA

Development and Evaluation of a Novel Inhalable Liposomal Powder Co-Encapsulating ASSNAC and Pirfenidone via Spray Freeze-Drying for Targeted Pulmonary Fibrosis Therapy. (PubMed, Pharmaceuticals (Basel)) - "S-Allylmercapto-N-acetylcysteine (ASSNAC) has been demonstrated to possess significant anti-inflammatory and antioxidant properties. Pulmonary administration avoids oral pirfenidone-induced liver and stomach toxicity. Collectively, the ASSNAC + PFD co-encapsulated liposomal dry powder produced by SFD represents a safe and efficacious therapeutic strategy for PF."

Journal • Fibrosis • Hematological Disorders • Immunology • Pulmonary Disease • Respiratory Diseases • TGFB1

Hepcidin as a Biomarker of Response to Antifibrotic Therapy in Idiopathic Pulmonary Fibrosis. (PubMed, J Clin Med) - "The change in hepcidin did not differ between the pirfenidone and nintedanib groups (p = 0.817). Our study provides the first real-world data demonstrating a significant decrease in serum hepcidin following antifibrotic treatment in patients with IPF. These findings suggest that serum hepcidin may have potential clinical utility as a biomarker for assessing response to antifibrotic therapy in IPF."

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

A Comparative Assessment of the Antifibrotic Effect of Nintedanib Administered via a Medicated Diet or Oral Gavage in a Rat Model of Bleomycin-Induced Pulmonary Fibrosis. (PubMed, Int J Mol Sci) - "Pirfenidone and nintedanib slow lung function decline, and the PDE4B inhibitor nerandomilast and treprostinil have recently shown promise as next-generation treatments. In conclusion, nintedanib retained robust antifibrotic activity when administered via dietary supplementation despite lower, but continuous, active plasma concentrations. Collectively, these findings indicate that optimizing drug delivery and absorption kinetics may achieve sustained therapeutic efficacy while reducing unnecessary exposure to supratherapeutic concentrations, thereby potentially improving tolerability."

Biomarker • Clinical • Journal • Preclinical • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • FGF2 • MMP7

Parameters predicting mortality in antifibrotic-treated IPF patients: mMRC, CPI, TLCO, FVC, GAP. (PubMed, Biomark Med) - "In the multivariate regression analysis, the following were identified as independent predictors of increased mortality risk: Symptom progression (Hazard Ratio(HR)=1.64, 95%Confidence Interval (CI):1.13-2.37, p = 0.008), mMRC >2 (HR = 1.62, 95%CI:1.13-2.32, p = 0.009), presence of radiologically honeycombing (HR = 1.91, 95%CI:1.21-3.02, p = 0.006), CPI >49.2 (HR = 1.60, 95% CI:1.15-2.22, p = 0.005), FVC ≤71% (HR = 1.41, 95% CI:1.00-1.99, p = 0.048). Patients with symptom progression, mMRC >2, presence of honeycombing on radiological imaging, FVC ≤71%, and CPI >49.2 have a higher risk of mortality and should be referred for pulmonary rehabilitation and lung transplantation evaluation at an early stage."

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

Nanocarrier-Enabled siRNA Therapy for Pulmonary Fibrosis: Pharmacological Rationale, Delivery Barriers, and Translational Opportunities. (PubMed, Int J Nanomedicine) - "Nintedanib and pirfenidone slow functional decline but do not reverse established fibrosis. We also consider single-target and multitarget strategies, pulmonary and systemic administration, disease-model limitations, and early clinical experience. The resulting target-to-delivery framework defines the evidence required before pulmonary siRNA nanomedicines can enter informative clinical testing."

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

Safety and Tolerability of Pirfenidone in Acute Pancreatitis (clinicaltrials.gov) - P1/2 | N=1 | Completed | Sponsor: University of Alabama at Birmingham | Recruiting ➔ Completed | N=60 ➔ 1 | Trial completion date: Feb 2027 ➔ Feb 2026

Enrollment change • Trial completion • Trial completion date • Pancreatitis • CXCL8

Late Breaking Abstract - OGG1 organocatalytic switches attenuate fibrotic remodelling in patient-derived fibrotic lung slices (ERS 2026) - "Nintedanib and pirfenidone were included as reference antifibrotic comparators. OGG1 organocatalytic switching identifies candidate antifibrotic compounds in patient-derived fibrotic lung tissue. These data support ORCAs as a novel therapeutic concept for progressive fibrotic ILD/DPLD. *ChatGPT used for drafting; authors reviewed/edited and take responsibility."

Clinical • Late-breaking abstract • Fibrosis • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Real-world safety of nintedanib and pirfenidone (ERS 2026) - "Overall, the pattern of adverse events was consistent with those reported in pivotal clinical trials, while the observed discontinuation rates highlight the importance of tolerability in routine clinical practice. Larger real-world studies are warranted to further explore comparative tolerability."

Clinical • Real-world • Real-world evidence • Fibrosis • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

COMPARATIVE EFFECTIVENESS OF PIRFENIDONE, NINTEDANIB, AND NO ANTIFIBROTIC THERAPY IN IDIOPATHIC PULMONARY FIBROSIS: A PROPENSITY SCORE-MATCHED ANALYSIS OF 10-YEAR MORTALITY (CHEST 2026) - No abstract available

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

Patient-reported adverse events and management of antifibrotic therapy in IPF: A multicenter, cross-sectional real-world study. (PubMed, Ther Adv Respir Dis) - "Adverse events and their management were compared between pirfenidone and nintedanib users.ResultsAmong 470 patients, 50.6% used pirfenidone and 49.4% nintedanib. Female sex and the presence of comorbidities were associated with a higher frequency of certain adverse events. Supportive treatment and dose adjustments are crucial for maintaining adherence."

Adverse events • Clinical • Journal • Real-world evidence • Anorexia • Fatigue • Idiopathic Pulmonary Fibrosis

CON: not so fast – upfront combination therapy is not yet ready for routine practice (ERS 2026) - "While antifibrotic therapies such as nintedanib and pirfenidone have demonstrated efficacy in slowing disease progression, robust data supporting routine upfront combination therapy remain limited. Emerging therapies, including nerandomilast, have shown promising results in recent trials, but questions remain regarding optimal treatment sequencing, long-term safety, tolerability and cost-effectiveness when used in combination. This presentation will critically review the available evidence, highlight the gaps that still need to be addressed through dedicated clinical trials, and argue that a cautious, stepwise treatment approach remains the most appropriate strategy in current clinical practice for patients with fibrotic interstitial lung disease."

Combination therapy • Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Disease • Respiratory Diseases

Late Breaking Abstract - Slow-Releasing Hydrogen Sulphide Donor GYY4137, Alone or in Combination with Pirfenidone, Attenuates Bleomycin-Induced Pulmonary Fibrosis in Mice (ERS 2026) - "These findings extend our previous observations on i.n. GYY4137 treatment and suggests a superior option to PIR for consideration as an add-on therapy. This work has been supported by the Scientific Research Projects Coordination Unit of Hacettepe University under grant number TOA-2025-21834."

Combination therapy • Late-breaking abstract • Preclinical • Fibrosis • Immunology • Inflammation • Interstitial Lung Disease • Respiratory Diseases

Macrophage-fibroblast communication driven by biomechanical stress in the pulmonary profibrotic niche: From signalling transduction to clinical translation. (PubMed, Cells Dev) - "The three approved antifibrotic agents - pirfenidone, nintedanib, and nerandomilast - receive only moderate endorsements in current guidelines, as they decelerate rather than arrest or reverse fibrotic remodelling, and none directly engages the mechanical dysregulation of the extracellular matrix (ECM) that underlies fibrogenesis. Finally, we discuss the clinical translation of mechanosignalling-targeted antifibrotic therapies, from the limited mechanosensing engagement of approved drugs to current pipeline advances and the preclinical and clinical hurdles ahead. By framing fibrosis as a druggable biomechanical circuit, this review aims to inform strategies that halt - and potentially reverse - pulmonary fibrosis."

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

PRO: upfront combination therapy in pulmonary fibrosis – start early, target more (ERS 2026) - "Building on current evidence with antifibrotic agents such as nintedanib, pirfenidone and nerandomilast, the presentation will discuss the limitations of monotherapy and the biological rationale for simultaneously targeting multiple profibrotic pathways. Drawing parallels with treatment strategies successfully implemented in other chronic conditions, such as pulmonary arterial hypertension, this presentation will argue that an upfront combination approach could be an effective next step in improving outcomes for patients with fibrotic interstitial lung diseases. Evidence, potential benefits and remaining challenges will be critically examined to support the case for a paradigm shift towards earlier, multitarget treatment strategies."

Combination therapy • Cardiovascular • Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Randomised controlled trial of partitioned aerobic exercise training using one-leg cycling in patients with idiopathic pulmonary fibrosis. (PubMed, Thorax) - P=N/A | "Partitioning training to a smaller muscle volume (one-leg) improved exercise endurance more than two-leg cycle training in patients with stable moderate-to-severe IPF."

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

Comparative serum proteomics of idiopathic pulmonary fibrosis patients treated with pirfenidone or nintedanib (ERS 2026) - "The circulating proteome of IPF patients reveals persistent epithelial injury and ECM turnover that remain largely unchanged under antifibrotic therapy. These findings suggest that pirfenidone and nintedanib stabilize rather than reverse disease progression and identify circulating proteins as potential stable biomarkers of IPF disease activity."

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

Gastrointestinal and skin safety evaluation of pirfenidone versus nintedanib: an analysis of real-world pharmacovigilance and randomized controlled trials. (PubMed, Naunyn Schmiedebergs Arch Pharmacol) - "Both drugs primarily caused gastrointestinal ADRs (e.g., nausea, vomiting), with pirfenidone linked to more photosensitivity reactions. Integrated pharmacovigilance and RCT analyses highlight the safety profiles of pirfenidone and nintedanib, offering evidence for clinical decision-making in idiopathic pulmonary fibrosis (IPF) treatment."

Adverse events • Journal • Real-world evidence • Gastroenterology • Gastrointestinal Disorder • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

Baseline Clinical and Functional Spectrum of Idiopathic Pulmonary Fibrosis Patients in a Fully Enrolled Global Phase 2 Trial of the PRS Inhibitor Bersiporocin (ERS 2026) - P2 | "Participants were randomized 2:1 to receive bersiporocin 150 mg twice daily or placebo for 24 weeks and stratified by background antifibrotic use (pirfenidone or nintedanib vs no antifibrotics). In this fully enrolled Phase 2 trial of bersiporocin, mean baseline FVC was numerically higher in patients not receiving background antifibrotics. Similar trends have been noted in recent IPF trials by baseline antifibrotic use. These baseline data provide context for planned subgroup interpretation of efficacy and safety outcomes by treatment status."

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

COMPARATIVE OUTCOMES OF PIRFENIDONE VS NINTEDANIB IN FIBROTIC LUNG DISEASE (CHEST 2026) - No abstract available

Fibrosis • Pulmonary Disease • Respiratory Diseases

Clinical efficacy of pharmacological treatments for idiopathic pulmonary fibrosis: a systematic literature review (ERS 2026) - "Eighteen trials assessed pirfenidone (PIR) or nintedanib (NIN) as monotherapy...By treatment group, mCFB values were −30.9 to −95 mL with NIN 150 mg twice daily (NIN-150BID); −115 mL with nerandomilast 18 mg; −235 mL with PIR 2403 mg/day; and −183.5 to –428 mL with placebo (PBO)...Acute exacerbation rates over 52 weeks ranged 0%–33% across 7 trials, with NIN-150BID, 0%–6.1%; NIN-150BID + PIR 1200–1800 mg/day, 33%; PIR 2403 mg/day, 4%; PIR 2403 mg/day + sildenafil 60 mg/day, 11%; and PBO, 1.8%–15.7%... Variable efficacy of current therapies on lung function and acute exacerbations highlights the unmet need for better IPF therapies."

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

Reconfiguring the macrophage-centric intercellular network in pulmonary fibrosis: Emerging perspectives and therapeutic opportunities. (PubMed, Pharmacol Res) - "Currently, only nintedanib, pirfenidone, and the novel phosphodiesterase 4B (PDE4B) inhibitor nerandomilast have received regulatory approval for clinical use. Emphasis is placed on reviewing the pharmacological mechanisms and recent advances in natural active compounds targeting this core cellular network to intervene in PF. The objective is to offer a theoretical foundation for a more comprehensive understanding of PF pathogenesis and to facilitate the optimization and innovation of anti-fibrotic therapeutic strategies."

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

Baseline quantitative computed tomography (CT) scores predict progression in patients with idiopathic pulmonary fibrosis (IPF): data from the FIBRONEER-IPF trial (ERS 2026) - " A total of 370 patients had baseline QLF and QILD scores, of whom 76.8% took nintedanib or pirfenidone. These results support the use of quantitative CT, particularly QLF score, to identify patients with IPF at higher risk of short-term progression."

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

Antifibrotic Use in Hypersensitivity Pneumonitis: Real-World Data from a Subgroup Analysis of the TURK-HP Cohort (ERS 2026) - "Pirfenidone and nintedanib were used in 5.1% and 12.9%, respectively (~18% overall). Approximately one-fifth of HP patients received antifibrotics, mainly those with fibrotic/UIP features. Prospective studies are needed to clarify selection and long-term outcomes in HP-related PPF."

Clinical • Real-world • Real-world evidence • Fibrosis • Immunology • Inflammation • Interstitial Lung Disease • Pneumonia • Pulmonary Disease

BEYOND EFFICACY: A SYSTEMATIC REVIEW AND META-ANALYSIS OF TOLERABILITY AND TREATMENT PERSISTENCE WITH PIRFENIDONE VS NINTEDANIB IN IDIOPATHIC PULMONARY FIBROSIS (CHEST 2026) - No abstract available

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

Predictors of anti-fibrotic tolerance in idiopathic and progressive pulmonary fibrosis: Real world data from a UK specialist ILD centre (ERS 2026) - " We performed a retrospective cross-sectional study of ILD patients treated with either Pirfenidone or Nintedanib from the ILD clinic during 2020-2025. Younger age, higher BMI, and better lung function are positively associated with antifibrotics tolerance. Patients unable to tolerate antifibrotics had significantly higher all-cause mortality. These findings highlight key considerations when initiating antifibrotic therapy and emphasize the significance of antifibrotic to reduce the disease progression with an aim to survival benefit."

Clinical • Real-world • Real-world evidence • Anorexia • Cardiovascular • Idiopathic Pulmonary Fibrosis • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases