PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis

NCT06855329 | Recruiting

• 1 other identifier: STUDY00002006
• Study Type: observational
• Target: 500
• Locations: 5 countries
• Sites: 13

Brief Summary

This is a prospective, observational cohort study. Participants with non-idiopatic pulmonary fibrosis, interstitial lung disease (ILD) will be followed for 24 months to systematically collect clinical, imaging, and biospecimen data. The primary objective is to optimize progressive pulmonary fibrosis (PPF) classification and establish PPF incidence for key ILD subtypes. Additional exploratory objectives are to 1) Prospectively validate a novel PPF classifier and assess performance durability over time, and 2) Determine whether multi-dimensional PPF prediction outperforms component approaches.

Conditions

Progressive Pulmonary Fibrosis; Interstitial Lung Disease

Keywords

Progressive pulmonary fibrosis; Interstitial lung disease; PRIME; PRIME-PPF; PPF

Outcome Measures

Primary Outcomes (1)

• Transplant-free survival (TFS)

  TFS is defined as the time from the 12-month pulmonary function tests to death or lung transplant. Censoring will occur at 12-months or earlier if lost to follow-up.

  12-months to 24-months (following an observation period from 0 months to 12 months)

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients with non-idiopathic pulmonary fibrosis (non-IPF) fibrosing ILD, including connective tissue disease associated ILD (CTD-ILD), fibrotic hypersensitivity pneumonitis (fHP) and non-IPF idiopathic interstitial pneumonia (IIP).

You may qualify if:

• 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.

You may not qualify if:

• 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.

Sponsors & Collaborators

• University of Massachusetts, Worcester: lead
• University of Michigan: collaborator
• University of Virginia: collaborator
• Johannes Gutenberg University Mainz: collaborator
• Fondazione Policlinico Universitario Agostino Gemelli IRCCS: collaborator

Study Sites (13)

University of Massachusetts Chan Medical School

Worcester, Massachusetts, 01655, United States

RECRUITING

University of Michigan

Ann Arbor, Michigan, 48109, United States

RECRUITING

University of Texas Southwestern

Dallas, Texas, 75390, United States

NOT YET RECRUITING

University of Virginia

Charlottesville, Virginia, 22908, United States

NOT YET RECRUITING

University of Washington

Seattle, Washington, 98195, United States

NOT YET RECRUITING

Royal Prince Alfred Hospital

Camperdown, New South Wales, 2050, Australia

NOT YET RECRUITING

Prince Charles Hospital

Brisbane, Queensland, 4032, Australia

NOT YET RECRUITING

Austin Health

Melbourne, Victoria, 3000, Australia

NOT YET RECRUITING

University of Calgary

Calgary, Alberta, T2N 4Z6, Canada

NOT YET RECRUITING

University of British Columbia

Vancouver, British Columbia, V6Z 1Y6, Canada

NOT YET RECRUITING

University College Dublin

Dublin, Ireland, D04 T6F4, Ireland

NOT YET RECRUITING

Royal Brompton

London, Greater London, SW3 6NP, United Kingdom

NOT YET RECRUITING

University Hospital Southampton

Southampton, Hampshire, SO16 7DL, United Kingdom

NOT YET RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

Plasma, serum, DNA, RNA, nasal, buccal, and fecal swabs

MeSH Terms

Conditions

Lung Diseases, Interstitial

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases

Study Officials

• Fernando J Martinez, MD, MS

  University of Massachusetts Chan Medical School

  PRINCIPAL INVESTIGATOR

• Justin Oldham, MD, MS

  University of Michigan

  PRINCIPAL INVESTIGATOR

• Cathie Spino, DSc

  University of Michigan

  PRINCIPAL INVESTIGATOR

• Imre Noth, MD, MS

  University of Virginia

  PRINCIPAL INVESTIGATOR

• Michael Kreuter, MD

  Johannes Gutenberg University Mainz

  PRINCIPAL INVESTIGATOR

• Dinesh Khanna, MD, MS

  University of Michigan

  PRINCIPAL INVESTIGATOR

• Luca Richeldi, MD, PhD

  Policlinico Gemelli

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Fernando J Martinez, MD, MS

CONTACT

508-334-8685; fernando.martinez1@umassmed.edu

Elizabeth Peters, BSN

CONTACT

774-455-4432; elizabeth.peters2@umassmed.edu

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor

Study Record Dates

• First Submitted: February 25, 2025
• First Posted: March 3, 2025
• Study Start: May 1, 2025
• Primary Completion (Estimated): December 1, 2029
• Study Completion (Estimated): December 1, 2029
• Last Updated: September 22, 2025

Record last verified: 2025-09

Data Sharing

• IPD Sharing: Will not share

Locations

AU (3); US (5); CA (2); IE (1); GB (2)