Idiopathic Pulmonary Fibrosis
Conditions
Keywords
Idiopathic pulmonary fibrosis, IPF, Disease progression, Acute exacerbation, Antifibrotic therapy, Nintedanib, Pirfenidone, Real-world data, Machine learning, Prognostic model, External validation, Multicentre cohort
Brief summary
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with marked inter-individual heterogeneity in trajectories and outcomes. Despite antifibrotic therapies, reliable risk stratification in routine practice remains suboptimal. OPEN-IPF is a multicentre retrospective observational cohort study designed to build a harmonised real-world dataset across Italian IPF referral centres to enable the development and external validation of machine-learning (ML) models predicting clinically relevant outcomes.
Detailed description
OPEN-IPF addresses the current limitation of AI/ML research in IPF-namely, the lack of large multicentre real-world datasets with harmonised variables and robust external validation. The study will retrospectively include adult patients with IPF followed in routine practice in participating Italian referral centres from 1 January 2015 to 31 December 2025 (data lock). No study-specific procedures will be performed. De-identified/pseudonymised data will be collected using a common data model, including demographics, smoking history, comorbidities, pulmonary function (FVC, DLCO), oxygen requirement, 6-minute walk test (where available), antifibrotic treatment exposure, HRCT features routinely reported, basic laboratory parameters, and clinical outcomes. The primary modelling targets are disease progression, acute exacerbations of IPF (AE-IPF), and real-world response to antifibrotic treatment. Model development will be performed using multicentre data with explicit external validation across centres
Interventions
None listed
Sponsors
University of Modena and Reggio Emilia
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 90 Years
Healthy volunteers
No
Inclusion criteria
* Age ≥18 years * Diagnosis of idiopathic pulmonary fibrosis established according to international guidelines and local multidisciplinary team (MDT) assessment * Availability of baseline clinical and functional data * Availability of follow-up data for at least 12 months, or until a clinically relevant event (e.g., death, lung transplantation)
Exclusion criteria
* Interstitial lung disease other than IPF * Lung transplantation performed before the baseline (index) date * Absence of any follow-up information after baseline
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Disease progression (guideline-based functional/composite criteria) | From baseline (index date) up to 12 months and up to end of available follow-up (maximum: 31 December 2025) | Disease progression defined using guideline-based criteria derived from routinely collected clinical data (e.g., decline in lung function and/or composite progression definitions as per the shared operational document). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Acute exacerbation of IPF (AE-IPF) | From baseline to end of follow-up (maximum: 31 December 2025) | Occurrence of AE-IPF during follow-up, adjudicated from routine clinical documentation using standardised operational definitions shared across centres. |
| Real-world response to antifibrotic therapy | From treatment initiation (or baseline if already treated) up to 12 months and end of follow-up (maximum: 31 December 2025) | Treatment response assessed in routine clinical practice using longitudinal clinical/functional data and treatment exposure information (type, start, discontinuation) |
| Overall survival | From baseline to end of follow-up (maximum: 31 December 2025) | Time from baseline to death from any cause. |
| Transplant-free survival | From baseline to end of follow-up (maximum: 31 December 2025) | Time from baseline to lung transplantation or death. |
| Time to first progression or AE-IPF event | From baseline to end of follow-up (maximum: 31 December 2025) | Time from baseline to first occurrence of disease progression or AE-IPF. |
Contacts
CONTACTRoberto Tonelli, MD, PhD
CONTACTStefania Cerri, MD, PhD
Outcome results
None listed