About The Registry

What is the difference between a prospective registry and a clinical trial? What is the goal of this Registry? How has the Registry contributed to IPF and ILD research? Why is the Registry adding participants to a new cohort? What information is being collected for the IPF-PRO Expansion Cohort? How has the project evolved?

What is the difference between a prospective registry and a clinical trial?

A prospective registry is a type of observational study in which researchers collect and record specific information about participants over time to gather data on the natural history of diseases, the effectiveness of treatments, and the types of care provided to patients. Once enrolled in a registry, patients continue to receive usual medical care.

A clinical trial is a type of research study that tests how well new medical approaches work in people, including methods for screening, preventing, diagnosing, or treating a disease. Trials involve specific medication or other interventions, and participants may receive a study drug or placebo (which looks like the study drug but has no active ingredients and should have no effect.)

What is the goal of this Registry?

The Registry aims to improve understanding of the clinical course of IPF and ILD, the impact of these diseases on patients, and current practices in diagnosis and care.

The Registry also seeks to advance the understanding of IPF and ILD patient-centered outcomes, such as causes and extent of cough, quality of life, and disease progression as well as identify novel blood, genetic, and imaging biomarkers of disease and disease progression.

For a detailed overview of the Registry, please see ClinicalTrials.gov (NCT01915511).

How has the Registry contributed to IPF and ILD research?

Data from the IPF-PRO/ILD-PRO Registry has advanced our understanding of the natural history of the diseases, possible treatments, and quality of life for people living with IPF and ILD.

Contributions from Registry participants and researchers have enabled the discovery of IPF and ILD biomarkers that are linked to disease or disease progression.

Why is the Registry adding participants to a new cohort?

With advances in the care of patients with IPF and two approved treatments, there is a continued need for additional research to improve our understanding of the use of existing treatments, their potential long-term benefits, and the biomarkers of treatment response in a contemporary cohort of people with IPF. Therefore, the study is currently enrolling a 1,000 IPF patients into the new IPF PRO Expansion Cohort to improve our understanding of the clinical course of IPF and its impact on people in a modern era of treatment and imaging diagnostics.

Potential participants must be 21 or older and have a new diagnosis of IPF established by an enrolling center in the last 12 months. Exclusion criteria for potential participants include:

Malignancy, treated or untreated, other than skin cancer or early-stage prostate cancer, within the past 5 years
Currently listed for lung transplantation at the time of enrollment into the Registry
Currently enrolled in an interventional clinical trial at the time of enrollment in the Registry
Previous enrollment in the IPF-PRO Cohort

What information is being collected for the IPF-PRO Expansion Cohort?

The Registry is currently focused on collecting data to better understand the course of IPF, how it affects patients, and current practices in diagnosis and patient care. Like previous cohorts, clinical data, health-related quality of life data, and research biosamples are being collected for the expansion cohort. What is unique to the IPF-PRO Expansion Cohort is the collection of digital chest imaging (HRCT scans) and disease-specific health-related quality-of-life data instruments being used.

All of the data for the Registry is collected during usual clinical care visits or over the phone; no extra visits or treatments are required for Registry participants.

Participants are contacted twice a year by telephone at a time convenient for them to answer specific questions about their symptoms, exposures, quality of life, and any clinical changes.

How has the project evolved?

The project began in 2014 as a small project to collect data across 20 centers from 300 patients with IPF. The engagement and interest from the IPF community supported an expansion both in size and scope.

The Registry has grown extensively into a multimillion-dollar project, with studies focused on biomarkers research, image analysis, patient-centered outcomes, and treatment paradigms.

The Registry has had three distinct phases, or cohorts, with more than 2,000 participants enrolled across more than 40 sites.

The recently launched IPF-PRO Expansion Cohort is actively recruiting 1,000 new patients from a network of more than 40 academic and community practice ILD centers across the US, with enrollment starting in October 2024 and follow up for this cohort will continue through 2030.

Click on the boxes below to learn more about each data type being collected for the IPF-PRO Expansion Cohort:

Clinical Data Health-related Quality of Life (HRQoL) Data Research Biosamples

Clinical Data

Includes:
- Medical history
- Lung function
- Medication use
- Supplemental oxygen use
- Digital chest imaging (HRCT scans)
- Labs/vitals
- Encounters with the health system
- Clinically relevant events
Collected to improve understanding of the clinical course of these diseases and current practices in diagnosis and care in a real-world setting.
Abstracted from medical records retrospectively for the 12 months prior to enrollment and prospectively at 6-month intervals for the duration of the study.

Health-related Quality of Life (HRQoL) Data

Includes data related to how IPF and ILD impact participants’ overall health, including:
- Mental and physical health
- Daily activities
Collected to improve understanding of the impact of these diseases on participants’ daily lives
Surveys are completed at 6-month intervals during regular pulmonary clinic visits
Collected using:
- George’s Respiratory Questionnaire (SGRQ)
- Living with Pulmonary Fibrosis QuestionnairCough Numeric Rating Scale (Cough NRS)
- Patient Health Questionnaire-2 (PHQ-2)

Research Biosamples

ILD-PRO Cohort and ILD-PRO BIO

The ILD-PRO Cohort enrolled 1,000 progressive non-IPF ILD patients from a network of more than 40 academic and community practice ILD centers across the US from February 2019 through October 2024. Follow-up for this cohort will continue for 3 to 5 years.

The ILD-PRO BIO biosample repository consists of DNA, RNA, serum, and plasma samples collected approximately every 6 months from participants enrolled in the ILD-PRO cohort.

IPF-PRO Cohort and IPF-PRO BIO

The original IPF-PRO Cohort enrolled approximately 1,000 IPF patients from a network of more than 40 academic and community practice IPF centers across the US from June 2014 through March 2019. The study reached a major milestone in 2024 when the IPF-PRO database was completed for this cohort.

The IPF-PRO BIO biosample repository consists of DNA, RNA, serum, and plasma samples collected approximately every 6 months from participants enrolled in the IPF-PRO cohort.

For more information on the Registry or our research, please contact us at IPF-PRO@dm.duke.edu.