Trials Sponsors Conditions Drugs Pricing

Conditions

Familial Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis (IPF)Interstitial Lung Disease Due to Systemic Disease (Telomere Biology Disorder)Progressive Pulmonary Fibrosis Telomere Disease

Keywords

PROGRESSIVE PULMONARY FIBROSISINTERSTITIAL LUNG DISEASETELOMERE DISORDERFAMILIAL PULMONARY FIBROSISIDIOPATHIC PULMONARY FIBROSIS

Outcome Measures

Primary Outcomes (1)

annual relative FVC decline over the observation period
The annual relative decline is defined as the difference between the final and the initial FVC value divided by the initial value, and it will be calculated for each year and for the entire follow-up period.
Annual for 5 years

Secondary Outcomes (4)

relative reduced annual FVC decline when compared to FPF patients treated with immunomodulator agents and FPF patients not treated in a 5-years period of FU.
5 years
mortality
5 years

Study Arms (1)

Familial pulmonary fibrosis

Eligible participants will be adults (≥18 years) with ILD documented on HRCT and with a positive genetic test, defined as the presence of rare variants or susceptibility polymorphisms consistent with the diagnosis of FPF.

Eligibility Criteria

Age18 Years+

Sexall

Healthy VolunteersNo

Age GroupsAdult (18-64), Older Adult (65+)

Sampling MethodNon-Probability Sample

Study Population

Patients with ILDs discussed in the multidisciplinary discussion to define FPF. Cases discussed will be documented using a standard CRF that detailed complete medical history including genetic test, physical examination, laboratory test results, pulmonary function test (PFT) results and, eventually, lung biopsy results and/or bronchoalveolar lavage (BAL) results. Pre-MDD diagnoses will be based on the referring pulmonologist's diagnosis and current consensus classification for ILDs. Post-MDD diagnosis will be classified according to Ryerson confidence terminology, with "confident diagnosis" reserved for \>90% clinical likelihood or a provisional diagnosis which was categorized as "high confidence" (70-89% likelihood) or "low confidence" (51-69% likelihood). Patients with less than 50% diagnostic confidence level post-MDD will be categorized as "unclassifiable ILD".

You may qualify if:

Sponsors & Collaborators

Fondazione Policlinico Universitario Agostino Gemelli IRCCS
Othercollaborator
Ospedale San Paolo
Othercollaborator

Study Sites (1)

Francesco Amati

Rozzano, Italy, 20089, Italy

Related Publications (2)

Zhang D, Newton CA. Familial Pulmonary Fibrosis: Genetic Features and Clinical Implications. Chest. 2021 Nov;160(5):1764-1773. doi: 10.1016/j.chest.2021.06.037. Epub 2021 Jun 26.
PMID: 34186035BACKGROUND
Borie R, Kannengiesser C, Antoniou K, Bonella F, Crestani B, Fabre A, Froidure A, Galvin L, Griese M, Grutters JC, Molina-Molina M, Poletti V, Prasse A, Renzoni E, van der Smagt J, van Moorsel CHM. European Respiratory Society statement on familial pulmonary fibrosis. Eur Respir J. 2023 Mar 16;61(3):2201383. doi: 10.1183/13993003.01383-2022. Print 2023 Mar.
PMID: 36549714BACKGROUND

MeSH Terms

Conditions

Idiopathic Pulmonary FibrosisLung Diseases, Interstitial

Condition Hierarchy (Ancestors)

Pulmonary FibrosisLung DiseasesRespiratory Tract Diseases

Study Officials

Francesco Amati, MD
Humanitas Research Hospital IRCCS, Rozzano-Milan
PRINCIPAL INVESTIGATOR

Study Design

Study Type: observational
Observational Model: COHORT
Time Perspective: PROSPECTIVE
Target Duration: 5 Years
Sponsor Type: OTHER
Responsible Party: SPONSOR

Study Record Dates

First Submitted

April 20, 2026

First Posted

May 12, 2026

Study Start

April 1, 2026

Actual (reported)

Primary Completion

December 2034

Estimated

Planned date reported by the registry; this milestone is not confirmed as achieved.

Hereditary Influences on Pulmonary Fibrosis Trajectories

Brief Summary

Registry Overview

Trial Relationships

Study Timeline

Study Start

First Submitted

First Posted

Primary Completion

Study Completion

Conditions

Keywords

Outcome Measures

Primary Outcomes (1)

Secondary Outcomes (4)

Study Arms (1)

Familial pulmonary fibrosis

Eligibility Criteria

You may qualify if:

Sponsors & Collaborators

Study Sites (1)

Related Publications (2)

MeSH Terms

Conditions

Condition Hierarchy (Ancestors)

Study Officials

Study Design

Study Record Dates

You may not qualify if:

Source record

Locations

Brief Summary

Registry Overview

Trial Relationships

Related Scientific Literature

Study Timeline

Study Start

First Submitted

First Posted

Primary Completion

Study Completion

Conditions

Keywords

Outcome Measures

Primary Outcomes (1)

Secondary Outcomes (4)

Study Arms (1)

Familial pulmonary fibrosis

Eligibility Criteria

You may qualify if:

Sponsors & Collaborators

Study Sites (1)

Related Publications (2)

MeSH Terms

Conditions

Condition Hierarchy (Ancestors)

Study Officials

Study Design

Study Record Dates

You may not qualify if:

Source record

Locations