Baseline Quantitative Computed Tomography (CT) Scores Predict Progression in Patients with Idiopathic Pulmonary Fibrosis (IPF): Data from the FIBRONEER-IPF Trial

Presenter: Jonathan Goldin

Quantitative CT measures may help characterise disease burden in idiopathic pulmonary fibrosis (IPF), but their ability to predict subsequent progression remains incompletely defined. This analysis assessed baseline quantitative lung fibrosis (QLF) and quantitative interstitial lung disease (QILD) scores in 370 patients from the placebo group of the FIBRONEER-IPF trial, examining associations with FVC change at week 52 and clinical outcomes. Mean (SD) QLF and QILD scores were 15.0 (9.0) and 31.5 (13.8), respectively; 76.8% of patients took nintedanib or pirfenidone. Higher baseline QLF and QILD scores were associated with greater FVC decline at week 52, with estimated changes of −50.7 mL (95% CI: −82.9, −18.5; p=0.002) and −56.9 mL (95% CI: −102.7, −11.1; p=0.015), respectively, per unit higher log2 baseline score. Over ≈16 months, QLF >20% was associated with increased risk of acute IPF exacerbation, respiratory hospitalisation, or death (HR 1.90 [95% CI: 1.20, 3.02]; p=0.007) and death (HR 2.51 [1.18, 5.35]; p=0.017), whereas QILD >40% was not significantly associated with these outcomes. QLF therefore showed particular potential for identifying patients at higher risk of short-term progression.

Late Breaking Abstract - Treatment Use and Outcomes in Progressive Fibrosing ILD: Real World Findings from the INCHANGE Study of Nintedanib

Presenter: Katrin Esther Hostettler.

In progressive pulmonary fibrosis (PPF), real-world use of nintedanib may involve treatment modifications related to tolerability. The multicenter INCHANGE study assessed nintedanib treatment patterns and associated outcomes in adults with non-IPF PPF across Central and Eastern Europe. Among 158 patients followed for up to 52 weeks, 131 (82.9%) started at 150 mg BID and 27 (17.1%) at 100 mg BID. Treatment modification occurred in 85 (53.8%), most commonly involving dose reduction (56.5%) or discontinuation (49.4%); reported reasons included safety-relevant events (22.8%) and other adverse events (18.1%). Standard dosing was maintained in 63 (39.9%), while 95 (60.1%) received modified dosing. No hospitalizations occurred with standard dosing versus 11 (11.6%) with modified dosing. The composite outcome of exacerbation, hospitalization, or death was more frequent with modified dosing (20.0% vs 3.2%; OR 7.6; 95% CI 1.1–54.1). Overall, 10 deaths and 8 acute ILD exacerbations occurred, 5 requiring hospitalization. These findings indicate frequent treatment modification, largely related to tolerability, with poorer clinical outcomes observed among patients receiving modified dosing.

Prednisone Combined with Mycophenolate Mofetil is Effective for Patients with Idiopathic Non-Specific Interstitial Pneumonia

Presenter: Tao Chen.

Treatment options for idiopathic non-specific interstitial pneumonia (iNSIP) remain an area of therapeutic evaluation, particularly regarding the role of steroid-sparing immunosuppression. This phase 3 randomized controlled trial compared prednisone alone with prednisone combined with mycophenolate mofetil (MMF) or cyclophosphamide (CTX) in patients aged 18–75 years with iNSIP. Among 107 patients completing the trial, 39 received prednisone, 34 MMF, and 34 CTX. After 3 months, FVC increased by 142 ml with prednisone and 132 ml with MMF, while it decreased by 16 ml with CTX. DLCO% increased by 2.6% with prednisone, 1.3% with MMF, and 2.3% with CTX. HRCT improvement was observed in 59.1% of the MMF group and 57.7% of the prednisone group, compared with 46.7% with CTX. Overall, prednisone plus MMF showed more favourable efficacy measures than prednisone plus CTX over 3 months, supporting MMF as a potential combination option in iNSIP.

Survival in Progressive Pulmonary Fibrosis Associated to Autoimmune Diseases: Multicenter Study from NEREA Registry

Presenter: Cristina Matesanz Lopez.

Autoimmune interstitial lung disease (ILD) with progressive pulmonary fibrosis (PPF) is associated with substantial ILD-related mortality, prompting evaluation of factors that may influence survival. A longitudinal multicenter study from the NEREA registry analysed 158 patients with connective tissue disease–ILD, IPAF, or unclassifiable autoimmune ILD who met PPF criteria between 2005–2024. Immunomodulatory therapy was used in 70.3% of patients at PPF diagnosis and in 81% during follow-up. The ILD-related mortality rate was 6.2 (95% CI 4.5–8.5) per 100 patient-years. Mean survival was 4.8 years among untreated patients versus over 10 years among treated patients. Immunomodulatory therapy was independently associated with lower ILD-related mortality (SHR 0.35, 95% CI 0.19–0.66; p=0.001). Conversely, DLCO <60% and emphysema were independently associated with higher ILD-related mortality. Overall, the findings indicate that immunomodulatory therapy was associated with substantially better ILD-related survival in this autoimmune PPF cohort, while impaired DLCO and emphysema identified patients with poorer outcomes.

Changes in Respiratory Function Tests and Radiological Findings in Patients with Idiopathic Pulmonary Fibrosis (IPF) and their Effect on Mortality

Presenter: Gülistan Karadeniz.

Mortality remains substantial in idiopathic pulmonary fibrosis (IPF), with changes in respiratory function and radiological parameters potentially providing prognostic information. This study assessed whether respiratory function tests and radiological measures at diagnosis and at 3 years predicted 3-year mortality in 387 IPF patients receiving antifibrotic treatment between January 1, 2013, and January 1, 2025. Within 3 years, 134 patients (35%) died. On ROC analysis, 3rd year TLCO<45% (AUC=0.690; p<0.001), 3rd year FVC<75% (AUC=0.678; p<0.001), TLCO at diagnosis<38% (AUC=0.670; p<0.001), FVC at diagnosis <75% (AUC=0.670; p<0.001), PA/Aorta ratio >0.86 at diagnosis (AUC=0.612; p<0.001), and 3rd year PA/Aorta ratio >0.86 (AUC=0.604; p=0.001) predicted mortality. Aortosternal distance did not predict mortality. Multivariate analysis identified 3rd year FVC<75% (HR=2.45; 95% CI: 1.24–4.87; p=0.010), PA/Aorta ratio >0.86 at diagnosis (HR=1.96; 95% CI: 1.18–3.24; p=0.009), and history of attacks (HR=1.76; 95% CI: 1.17–2.65; p=0.007) as independent risk factors. These parameters may help identify IPF patients at higher risk of 3-year mortality who require closer monitoring and consideration of transplantation.

Clinical Outcomes of Antifibrotic Therapy in Fibrotic Lung Disease: A Retrospective Single Centre Review over Last 10 Years

Presenter: Hira Gul.

Long-term management of fibrotic lung disease is challenged by substantial mortality, functional decline, and treatment-related adverse effects, while comparative real-world data across mixed populations remain limited. This retrospective cohort study assessed outcomes among 137 patients with IPF and PF-ILD receiving nintedanib or pirfenidone at University Hospital Limerick from 2016–2025, with a median follow-up of 2.65 years. Mortality was 61% with pirfenidone and 53% with nintedanib, with improved survival among patients treated for >1 year. Rates of FVC, TLC, and DLCO decline were similar between therapies. Annual exacerbations decreased from 4.5 to 3.0 events, with fewer exacerbations reported among nintedanib-treated patients (14.6%). Nintedanib had 14% discontinuation and 29.2% therapy switching, mainly related to gastrointestinal and hepatic adverse effects, while dose reduction allowed treatment continuation in 21.7%. Overall, antifibrotic treatment was associated with reduced exacerbations and improved survival, with treatment tolerability managed through monitoring and dose adjustments.

ERS Congress 2026, 5-9 September, Barcelona, Spain