OPEN-IPF: Observational Prediction modEl for cliNical Outcomes in Idiopathic Pulmonary Fibrosis (OPEN-IPF)

Observational Prediction Model for Clinical Outcomes in Idiopathic Pulmonary Fibrosis: a Multicentre, ML-driven Study (OPEN-IPF)

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.

Study Overview

• Status: Not yet recruiting
• Conditions: Idiopathic Pulmonary Fibrosis

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

• Study Type: Observational
• Enrollment (Estimated): 1000

Contacts and Locations

• Study Contact: Name: Roberto Tonelli, MD, PhD; Phone Number: 0039059425934; Email: rtonelli@unimore.it
• Study Contact Backup: Name: Stefania Cerri, MD, PhD; Phone Number: 00390594225335; Email: stefania.cerri@unimore.it

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Adults with idiopathic pulmonary fibrosis diagnosed according to international guidelines and local multidisciplinary team assessment, followed in routine care at participating Italian referral centres.

Description

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

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Disease progression (guideline-based functional/composite criteria)	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).	From baseline (index date) up to 12 months and up to end of available follow-up (maximum: 31 December 2025)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Acute exacerbation of IPF (AE-IPF)	Occurrence of AE-IPF during follow-up, adjudicated from routine clinical documentation using standardised operational definitions shared across centres.	From baseline to end of follow-up (maximum: 31 December 2025)
Real-world response to antifibrotic therapy	Treatment response assessed in routine clinical practice using longitudinal clinical/functional data and treatment exposure information (type, start, discontinuation)	From treatment initiation (or baseline if already treated) up to 12 months and end of follow-up (maximum: 31 December 2025)
Overall survival	Time from baseline to death from any cause.	From baseline to end of follow-up (maximum: 31 December 2025)
Transplant-free survival	Time from baseline to lung transplantation or death.	From baseline to end of follow-up (maximum: 31 December 2025)
Time to first progression or AE-IPF event	Time from baseline to first occurrence of disease progression or AE-IPF.	From baseline to end of follow-up (maximum: 31 December 2025)

Collaborators and Investigators

• Sponsor: University of Modena and Reggio Emilia

Study record dates

Study Major Dates

• Study Start (Estimated): June 1, 2026
• Primary Completion (Estimated): January 1, 2027
• Study Completion (Estimated): June 1, 2027

Study Registration Dates

• First Submitted: February 4, 2026
• First Submitted That Met QC Criteria: February 4, 2026
• First Posted (Actual): February 11, 2026

Study Record Updates

• Last Update Posted (Actual): February 11, 2026
• Last Update Submitted That Met QC Criteria: February 4, 2026
• Last Verified: February 1, 2026

More Information

Terms related to this study

• Keywords: Pirfenidone; External validation; Idiopathic pulmonary fibrosis; Acute exacerbation; Disease progression; Nintedanib; IPF; Machine learning; Real-world data; Prognostic model; Antifibrotic therapy; Multicentre cohort
• Additional Relevant MeSH Terms: Pathologic Processes; Disease Attributes; Respiratory Tract Diseases; Lung Diseases; Lung Diseases, Interstitial; Pulmonary Fibrosis; Pathological Conditions, Signs and Symptoms; Disease Progression; Idiopathic Pulmonary Fibrosis
• Other Study ID Numbers: UModenaReggio20262

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No