Following children and adults with interstitial lung disease and their relatives
Collects clinical data over time from people with lung scarring disease and relatives.
Plain-language title generated from the registry record
Registry title: Interstitial Lung Disease: A Study From Infancy to Elderly Including Relatives
Key facts
- Where
- Paris, FranceSee the site
AI summary: who the study is looking for
From the trial's eligibility criteria. Machine-read from the registry text on this page, so it can be wrong. The registry text below and the trial team decide who can take part.
The trial is looking for children and adults with certain scarring lung diseases (interstitial lung disease). First-degree relatives of people with a telomere gene change can also join.
- Sex
- All sexes can join.
- Disease stage
- Confirmed idiopathic interstitial pneumonia, or progressive fibrotic lung disease other than IPF, or lung disease linked to systemic sclerosis. For progressive and systemic sclerosis disease, scarring of at least 10% of the lungs on CT scan.
- Earlier treatment
- For progressive fibrotic disease, the disease must have kept worsening despite appropriate management over up to 24 months.
- Biomarker or test result
- Relatives can join if their first-degree relative carries a mutation in TERT, TERC, RTEL1, TINF2, DKC1, PARN or another telomere-related gene.
Main reasons for not taking part
- Worsening that is caused by pulmonary embolism, decompensated heart failure or a lower respiratory tract infection (for progressive fibrotic disease)
Read by machine on 12 November 2026.
Where in France
- RaDiCo-ILD2Paris, FranceContact: Sonia Gueguen, PHDTaking people
Questions to ask the study team
- How often do I need to come in, and for how long each time?
- Are travel costs paid?
- Can I keep my own doctor?
- What happens if I want to stop?
Contact the study team: see the registry record → Contacts and Locations
Print a page for my doctorTrial details
- Who runs it
- Institut National de la Santé Et de la Recherche Médicale, France
- Trial code
- NCT06036719
- Record updated
- 12 February 2026
- Original description
The concerned patients are children and adults suffering from idiopathic interstitial pneumonias, other chronic fibrosing interstitial pneumonias with a progressive phenotype, and interstitial pneumonia associated with Scleroderma and related cases of patients carrying a mutation on one of the telomere-associated genes.Read full original descriptionShow less
Interstitial Lung Disease: A Study From Infancy to Elderly Including Relatives
The concerned patients are children and adults suffering from idiopathic interstitial pneumonias, other chronic fibrosing interstitial pneumonias with a progressive phenotype, and interstitial pneumonia associated with Scleroderma and related cases of patients carrying a mutation on one of the telomere-associated genes.
This is a national, observational, longitudinal, multicenter study that will be conducted retrospectively and prospectively. It aims to collect consistent and comparable clinical data for patients and their relatives, whether they carry a mutation or not, affected by diffuse idiopathic interstitial pneumopathy.
The expected duration of the study, including data analysis, is approximately 10 years (5 years for participant enrollment and 5 years of follow-up, in addition to the steps for data management and statistical analyses).
Each participating center will inform every participant by providing an information sheet, and their written consent will be obtained before including them in the study and commencing data collection.
Prospective medical data will be collected at 6 months to 1 year after enrollment and then at least once per year for patients up to 5 years and 5 years for their relatives.
Participants will complete a self-questionnaire during their regular follow-up consultations or by accessing a secure interface.
Inclusion: Confirmed diagnosis of IIP established based on clinical, radiological, or functional criteria; Confirmed diagnosis of non-IPF progressive fibrotic interstitial lung disease (PF-ILD) with fibrosis ≥ 10% on CT scan, disease worsening not related to pulmonary embolism, decompensated heart failure, or lower respiratory tract infection, and disease progression despite "appropriate management" evaluated over a period of up to 24 months:; A relative decline in Forced Vital Capacity (FVC) of at least 10% from predicted value, with or without clinical deterioration, or; A combination of at least 2 of the following criteria: a relative decline in FVC between 5% and 10% from predicted value, worsening respiratory symptoms, increased extent of pulmonary fibrosis on thoracic CT scan; Confirmed diagnosis of Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD) (American College of Rheumatology criteria), with a total score ≥ 9 and disease extent involving ≥ 10% of the lungs (defined by reticular abnormalities, honeycombing, and ground-glass opacities) on high-resolution CT (HRCT) scan; For relatives: First degree relatives of patients carrying a mutation in TERT, TERC, RTEL1, TINF2, DKC1, PARN genes, and other telomere related genes that may be described in the future and included.