Towards Standardisation in IPF / PPF Registry Data
This project is stage 1 of the flagship project: Towards Standardisation in IPF / PPF Registry Data: A global initiative. This standardisation tool will unify idiopathic pulmonary fibrosis (IPF)/progressive pulmonary fibrosis (PPF) data collection and analysis worldwide, standardising key variables and disease outcomes, to improve patient management, foster collaborative research, and accelerate the development of treatments.
Project 1: Identifying Key Variables through a Global Prioritisation Task
Objective
This project will work with international IPF/PPF experts and patient organisations to conduct a priority setting study with the aim to:
- Identify relevant diagnostic, clinical and patient-reported variables associated with multiple outcomes in idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) for harmonisation across global clinical registries.
- Determine essential data elements for standardisation across clinical registries to ensure consistency and comparability of data collection practices.
- Develop a registry standardisation tool to guide harmonised data collection globally.
- Explore similarities and differences in diagnostic and prognostic priorities across different regions.
Rationale
Interstitial lung diseases (ILDs) are a complex group of diseases which cause inflammation and scarring of the lungs, often irreversible and progressive such as IPF and PPF.1,2 Managing the diseases effectively requires a thorough understanding of their clinical features, disease and treatment outcomes.
There are several IPF/ILD registries, however the data which is collected varies widely,3-8 with differences in what data elements, definitions, and outcomes are recorded, making it difficult to compare datasets and generalise findings.9 As a result, current studies have not been able to consider the whole range of clinical variables relevant to IPF and PPFs.10
Inconsistencies in the quality and completeness of data add significant challenges in utilising the data effectively.11 Standardising the data collected in ILD registries is vital for improving the quality of data and enabling meaningful comparisons across registries.
Statement of Need
Standardising registries will enable researchers and clinicians to collate and analyse global data more effectively, increasing sample size and more robust evidence to guide clinical decision-making. International IPD/PPF experts have come together in a working group meeting to highlight the need for a robust and standardised approach for collecting global data in IPF/PPF registries, paving the way for thorough research, advancing our understanding of the disease and its stages and guiding future clinical decision-making.
Study Design
This project will use a priority setting exercise to identify the key variables for standardising data in IPF/PPF registries. The questions and statements used in the priority setting exercise will be developed by IPF/PPF experts and patient representatives within the study steering committee and ILD patient organisations under the supervision of the Principal Investigator.
Questions and statements in the exercise will focus on disease outcomes, treatment responses, diagnostic/prognostic tests, and assess the general priorities for standardising data, such as cost, patient acceptability, reproducibility, and required expertise/technology.
The defined questions and statements will be presented in an online application for clinical experts and patients to complete the priority setting exercise. The online application will conduct a Multi-Criteria Decision Analysis (MCDA) and employ a pairwise ranking approach.
Through the priority setting exercise and subsequent analysis, we will identify the most relevant diagnostic/prognostic tools, disease outcomes and essential data elements that should be captured in all IPF/PPF registries. This will form the first iteration of a registry standardisation tool to guide the harmonisation of data across all IPF/PPF registries.
Principal Investigator
Pilar Rivera-Ortega
Steering committee:
Rayid Abdulqawi, Gina Amanda, Katerina Antoniou, Azuma Arata, Milind Baldi, María Asunción Nieto Barbero, Ahmed Bayoumy, Jürgen Behr, Elisabeth Bendstrup, Guadalupe Bermudo, Demosthenes Bouros, Jack Callum, Nazia Chaudhuri, Vincent Cottin, Tamera Corte, Juan Enghelmayer, Helen Jo, Michael Keane, Martin Kolb, Andrew Li, Graham Lough, George Margaritopoulos, Fernando J Martinez, Cristina Matesanz-López, Maria Molina Molina, Steven D. Nathan, Laurence Pearmain, Lucilla Piccari, Ganesh Raghu, Claudia Ravaglia, Elisabetta Renzoni, Paola Rottoli, Abdullah Sakkat, Moises Selman, Jacabo Sellares, Stefan C. Stanel, Jeffrey Swigris, Gabriela Tabaj, Claudia Valenzuela, Brenda Varela, Eirini Vasarmidi, Athol Wells, plus additional patient representatives (+patients involved research design).
Study Status
This study is looking to secure the remaining 50% of the project budget
Study registration
TBA
Funding
TBA