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

September 16, 2025 updated by: Fernando J Martinez, University of Massachusetts, Worcester

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

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.

Study Overview

Status

Recruiting

Conditions

Detailed Description

This is a prospective, cohort study with no study investigational therapy prescribed. 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) will be recruited. Consented participants with non-IPF ILD will be carefully phenotyped with extensive clinical, physiological, and imaging data. After confirmation of eligibility, participants will undergo protocolized follow-up over 24 months. Clinical data and blood biospecimens will be collected at protocolized time points throughout the study.

The primary outcome is 12-month transplant-free survival (TFS), following a 12-month observation period with TFS defined as the time from 12-month PFT to death, lung transplant or censoring at 12 months or earlier if lost to follow-up. This research will determine whether a ≥10% relative decline in FVC occurring during the 12-month observation period is associated with TFS over the subsequent 12 months. The investigators will then explore other proposed PPF criteria using the same approach. Additionally, the investigators will determine test performance characteristics of a novel proteomic biomarker for predicting PPF at 12 months and compare performance of this classifier to a multi-dimensional approach that incorporates clinical data and quantitative CT data.

Study Type

Observational

Enrollment (Estimated)

500

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Adult
  • Older Adult

Accepts Healthy Volunteers

No

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).

Description

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.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Transplant-free survival (TFS)
Time Frame: 12-months to 24-months (following an observation period from 0 months to 12 months)

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)

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

University of Massachusetts, Worcester

Collaborators

Fondazione Policlinico Universitario Agostino Gemelli IRCCS
University of Michigan
University of Virginia
Johannes Gutenberg University Mainz

Investigators

  • Principal Investigator: Dinesh Khanna, MD, MS, University of Michigan
  • Principal Investigator: 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: Luca Richeldi, MD, PhD, Policlinico Gemelli

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

May 1, 2025

Primary Completion (Estimated)

December 1, 2029

Study Completion (Estimated)

December 1, 2029

Study Registration Dates

First Submitted

February 25, 2025

First Submitted That Met QC Criteria

February 25, 2025

First Posted (Actual)

March 3, 2025

Study Record Updates

Last Update Posted (Actual)

September 22, 2025

Last Update Submitted That Met QC Criteria

September 16, 2025

Last Verified

September 1, 2025

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • STUDY00002006

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

product manufactured in and exported from the U.S.

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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