PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis

ConditionsProgressive Pulmonary Fibrosis Interstitial Lung Disease

Trial statusRecruiting

Trial phaseNot listed

Trial typeObservational

Biological sexAll

Age18-80

SponsorUniversity of Massachusetts, Worcester

About this trial

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.

Eligibility criteria

Qualifiers

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.

Disqualifiers

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).