PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis

NCT ID: NCT06855329

Last Updated: 2025-09-22

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Total Enrollment

500 participants

Study Classification

OBSERVATIONAL

Study Start Date

2025-05-01

Study Completion Date

2029-12-31

Brief Summary

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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.

Detailed Description

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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.

Conditions

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Progressive Pulmonary Fibrosis Interstitial Lung Disease

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Eligibility Criteria

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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.
Minimum Eligible Age

18 Years

Maximum Eligible Age

80 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University of Michigan

OTHER

Sponsor Role collaborator

University of Virginia

OTHER

Sponsor Role collaborator

Johannes Gutenberg University Mainz

OTHER

Sponsor Role collaborator

Fondazione Policlinico Universitario Agostino Gemelli IRCCS

OTHER

Sponsor Role collaborator

University of Massachusetts, Worcester

OTHER

Sponsor Role lead

Responsible Party

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Fernando J Martinez

Professor

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Fernando J Martinez, MD, MS

Role: PRINCIPAL_INVESTIGATOR

University of Massachusetts Chan Medical School

Justin Oldham, MD, MS

Role: PRINCIPAL_INVESTIGATOR

University of Michigan

Cathie Spino, DSc

Role: PRINCIPAL_INVESTIGATOR

University of Michigan

Imre Noth, MD, MS

Role: PRINCIPAL_INVESTIGATOR

University of Virginia

Michael Kreuter, MD

Role: PRINCIPAL_INVESTIGATOR

Johannes Gutenberg University Mainz

Dinesh Khanna, MD, MS

Role: PRINCIPAL_INVESTIGATOR

University of Michigan

Luca Richeldi, MD, PhD

Role: PRINCIPAL_INVESTIGATOR

Policlinico Gemelli

Locations

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University of Massachusetts Chan Medical School

Worcester, Massachusetts, United States

Site Status RECRUITING

University of Michigan

Ann Arbor, Michigan, United States

Site Status RECRUITING

University of Texas Southwestern

Dallas, Texas, United States

Site Status NOT_YET_RECRUITING

University of Virginia

Charlottesville, Virginia, United States

Site Status NOT_YET_RECRUITING

University of Washington

Seattle, Washington, United States

Site Status NOT_YET_RECRUITING

Royal Prince Alfred Hospital

Camperdown, New South Wales, Australia

Site Status NOT_YET_RECRUITING

Prince Charles Hospital

Brisbane, Queensland, Australia

Site Status NOT_YET_RECRUITING

Austin Health

Melbourne, Victoria, Australia

Site Status NOT_YET_RECRUITING

University of Calgary

Calgary, Alberta, Canada

Site Status NOT_YET_RECRUITING

University of British Columbia

Vancouver, British Columbia, Canada

Site Status NOT_YET_RECRUITING

University College Dublin

Dublin, Ireland, Ireland

Site Status NOT_YET_RECRUITING

Royal Brompton

London, Greater London, United Kingdom

Site Status NOT_YET_RECRUITING

University Hospital Southampton

Southampton, Hampshire, United Kingdom

Site Status NOT_YET_RECRUITING

Countries

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United States Australia Canada Ireland United Kingdom

Central Contacts

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Fernando J Martinez, MD, MS

Role: CONTACT

508-334-8685

Elizabeth Peters, BSN

Role: CONTACT

774-455-4432

Facility Contacts

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Roberto Caricchio, MD

Role: primary

Betsy Peters, BSN

Role: backup

774-455-4432

Janelle Pugashetti, MD

Role: primary

Nisha Mohan

Role: backup

Chad Newton, MD

Role: primary

214-645-6493

Nighat Sultana, MBBS

Role: backup

214-645-6605

Tessy Paul, MD

Role: primary

434-243-2398

Yousef Althulth

Role: backup

Ganesh Raghu, MD

Role: primary

(206) 598-4967

Katherine Palaeologus

Role: backup

(206)843-0228

Tamera Corte, MBBS, PhD

Role: primary

+61 (0)2 9515 6120

Jessica Rhodes

Role: backup

+61 (0) 2 9515 5006

John Mackintosh, MD

Role: primary

+617 3139 4000

Brittany Cooper

Role: backup

+617 3139 7222

Yet Khor, MD

Role: primary

+613 9596 5000

Lisa Fuhmeister

Role: backup

+613 9596 3874

Kerri Johannson, MD, MPH

Role: primary

403-220-2708

Curtis Dumonceaux

Role: backup

403-220-2123

Chris Ryerson, MD

Role: primary

1(604)258-8682

Miriam Margari

Role: backup

1(236)808-0804

Cormac McCarthy, MD, PhD

Role: primary

+35312213323

Gillian Buckley

Role: backup

+353012214000

Philip Molyneaux, MD

Role: primary

020 7352 8121

Role: backup

020 7352 8121

Sophie Fletcher, BM BCh

Role: primary

023 8120 4391

Matt Harvey

Role: backup

02381 205234

Other Identifiers

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STUDY00002006

Identifier Type: -

Identifier Source: org_study_id

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