Observational study on progressive pulmonary fibrosis and its classification

PRIME-PPF: PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis

This study is trying to better understand and classify progressive pulmonary fibrosis in people with certain lung diseases by following them over two years and collecting their health information and blood samples.

Quick facts

• Study type: Observational
• Enrollment: 500 (estimated)
• Ages: 18 Years to 80 Years
• Sex: All
• Sponsor: University of Massachusetts, Worcester (other)
• Locations: 13 sites (Worcester, Massachusetts and 12 other locations)
• Trial ID: NCT06855329 on ClinicalTrials.gov

What this trial studies

This observational cohort study follows participants with non-idiopathic pulmonary fibrosis and interstitial lung disease over 24 months to collect clinical, imaging, and biospecimen data. The primary goal is to optimize the classification of progressive pulmonary fibrosis (PPF) and establish its incidence among key subtypes of interstitial lung disease. Participants will undergo systematic phenotyping and will have their clinical data and blood biospecimens collected at designated time points. The study will also validate a novel PPF classifier and compare its performance to multi-dimensional prediction approaches.

Who should consider this trial

Good fit: Ideal candidates are adults aged 18-80 with a diagnosis of non-idiopathic fibrosing interstitial lung disease.

Not a fit: Patients with fibrosing interstitial lung disease diagnosed more than 5 years prior or with minimal ILD may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to improved classification and understanding of progressive pulmonary fibrosis, potentially enhancing patient management and outcomes.

How similar studies have performed: Other studies have shown promise in similar approaches to classifying and predicting outcomes in pulmonary fibrosis, but this specific methodology is novel.

Eligibility criteria

Inclusion Criteria:

* Age 18-80 years with a diagnosis of non-IPF fibrosing ILD due to CTD-ILD, fHP, or non-IPF IIP based on central review
* Diagnosis of Fibrotic ILD as determined by site investigator.
* Willingness to comply with study procedures and follow-up.
* Provide written informed consent.

Exclusion Criteria:

* Site diagnosis of fibrosing ILD \>5 years prior to Visit 1 (Screening and Baseline Visit).
* Minimal ILD, defined as reticular opacities and/or ground-glass opacities without architectural distortion (traction bronchiolectasis/bronchiectasis or honeycombing) affecting \< 5% of the lung on centralized evaluation of HRCT at Visit 1 (Screening and Baseline Visit). High quality historical chest HRCT may be used if performed within 90 days prior to Visit 1.
* Extent of emphysema \>15% of total lung volume or greater than extent of fibrosis based on central, qualitative assessment of HRCT at Visit 1. High quality historical chest HRCT may be used if performed within 90 days prior to Visit 1.
* Active malignancy within one year prior to Visit 1 (except for non-melanoma skin cancer requiring local treatment).
* Inability to complete full PFT (spirometry and DLCO) at Visit 1. Historical PFT may be used if performed within 90 days prior to Visit 1.
* Taking nintedanib or nerandomilast at Visit 1.
* Pregnancy at screening or plans to become pregnant during follow-up.
* Participation in an interventional clinical trial for fibrotic ILD at the time of Visit 1, or receipt of an investigational drug within the previous 4 weeks of the enrollment visit (Visit 1) or 5 times the half-life, if longer.

Where this trial is running

Worcester, Massachusetts and 12 other locations

• University of Massachusetts Chan Medical School — Worcester, Massachusetts, United States (RECRUITING)
• University of Michigan — Ann Arbor, Michigan, United States (RECRUITING)
• University of Texas Southwestern — Dallas, Texas, United States (NOT_YET_RECRUITING)
• University of Virginia — Charlottesville, Virginia, United States (NOT_YET_RECRUITING)
• University of Washington — Seattle, Washington, United States (NOT_YET_RECRUITING)
• Royal Prince Alfred Hospital — Camperdown, New South Wales, Australia (NOT_YET_RECRUITING)
• Prince Charles Hospital — Brisbane, Queensland, Australia (NOT_YET_RECRUITING)
• Austin Health — Melbourne, Victoria, Australia (NOT_YET_RECRUITING)
• University of Calgary — Calgary, Alberta, Canada (NOT_YET_RECRUITING)
• University of British Columbia — Vancouver, British Columbia, Canada (NOT_YET_RECRUITING)
• University College Dublin — Dublin, Ireland, Ireland (NOT_YET_RECRUITING)
• Royal Brompton — London, Greater London, United Kingdom (NOT_YET_RECRUITING)
• University Hospital Southampton — Southampton, Hampshire, United Kingdom (NOT_YET_RECRUITING)

Study contacts

• Principal investigator: Fernando J Martinez, MD, MS — University of Massachusetts, Worcester
• Study coordinator: Fernando J Martinez, MD, MS

Conditions: Progressive Pulmonary Fibrosis, Interstitial Lung Disease, Progressive pulmonary fibrosis, Interstitial lung disease, PRIME, PRIME-PPF, PPF