Status:

RECRUITING

Interstitial Lung Disease: A Study From Infancy to Elderly Including Relatives

Lead Sponsor:

Institut National de la Santé Et de la Recherche Médicale, France

Conditions:

Interstitial Lung Diseases

Eligibility:

All Genders

Brief Summary

The concerned patients are children and adults suffering from idiopathic interstitial pneumonias, other chronic fibrosing interstitial pneumonias with a progressive phenotype, and interstitial pneumon...

Eligibility Criteria

Inclusion

  • Inclusion Criteria:
  • 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.

Exclusion

    Key Trial Info

    Start Date :

    January 19 2022

    Trial Type :

    OBSERVATIONAL

    Allocation :

    ESTIMATED

    End Date :

    March 19 2031

    Estimated Enrollment :

    3000 Patients enrolled

    Trial Details

    Trial ID

    NCT06036719

    Start Date

    January 19 2022

    End Date

    March 19 2031

    Last Update

    January 29 2025

    Active Locations (1)

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    1

    RaDiCo-ILD2

    Paris, Île-de-France Region, France, 75012