Clinical trial NCT04238871: RaDiCo PID Cohort (RaDiCo-ILD Cohort in English)
Official title: Idiopathic Interstitial Pneumopathy : Genetic and Environmental Determinants From Infancy to Elderly
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Idiopathic Interstitial Pneumopathy/Pneumopathy Interstitial Diffuse
Paediatric and Adult Patients
Lead sponsor: Institut National de la Santé Et de la Recherche Médicale, France
Sponsor class: OTHER_GOV
First posted: 2020-01-23
Enrollment: 2500
Sex: ALL
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: OTHER
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patient with a diagnosis of Pneumopathie Interstitielle Diffuse/ Idiopathic Interstitial Pneumonia diagnosis is established on presenting history, clinical, radiological and functional and if available pathological findings.

Brief summary: The main objective is to describe the phenotypic features of the paediatric and adult patients with Idiopathic Interstitial Pneumopathy/Pneumopathy Interstitial Diffuse (IIP/PID), at diagnosis and during the follow-up. These data will be critical for the description of the natural history of the various forms of IIP/PID.

Detailed description: The French RaDiCo-PID : Idiopathic Interstitial Pneumopathy registry is an ongoing observational prospective and retrospective cohort with longitudinal long-term follow-up includes pediatric and adult patients with Idiopathic Interstitial Lung Disease (ILD) from the reference and competence centers.

Eligibility criteria: Inclusion Criteria:

* Clinical criteria: chronic respiratory insufficiency manifestations including dyspnea/tachypnea, cough, and cyanosis during exercise or at rest
* Radiological criteria: characteristic chest High-Resolution Computed Tomography (HRCT) abnormalities including widespread ground glass or alveolar attenuation, reticulation often associated with traction bronchiectasis, and honeycombing
* Functional criteria: pulmonary function test abnormalities reflecting a restrictive pattern and including: loss of lung volume, vital capacity (VC), total lung capacity (TLC); reduction in the diffusion capacity of the lung for carbon monoxide (DLCO), gas exchange abnormalities, and altered ventilatory response to exercise
* Patients (parents/guardians for paediatric/patients) having given an informed consent to participate in the protocol
* Patients affiliated to the "Regime National d'Assurance Maladie"

Exclusion Criteria:

* Patients with diffuse parenchymal lung diseases caused by drug toxicity, immunodeficiency, proliferative disorders including histiocytosis, and metabolic disorders
* Patients (parents/guardians for paediatric patient) not able to approve/understand the protocol

Primary outcomes:
measure: The phenotypic description idiopathic lung disease
timeFrame: Up to 10 years
Secondary outcomes:
measure: Identify gene factors involved in disease initiation and progression
timeFrame: Up to 10 years
measure: Investigate the extent to which environmental and co-morbidity factors may influence disease severity and outcome
timeFrame: Up to 10 years
measure: Identify and validate biomarkers for disease diagnosis and progression
timeFrame: Up to 10 years
Other outcomes:
Locations:
city: Bron
country: France
facility: CHU Lyon - Hôpital Louis Pradel
city: Paris
country: France
facility: AP-HP - Hôpital Armand Trousseau
Study officials:
References:
citation: Cottin V, Gueguen S, Nunes H, Jouneau S, Crestani B, Bonniaud P, Wemeau L, Israel-Biet D, Reynaud-Gaubert M, Gondouin A, Cadranel J, Marchand-Adam S, Chevereau M, Dufaure-Gare I, Amselem S, Clement A; and the RaDiCo team. Treatment of Idiopathic Pulmonary Fibrosis with Capsule or Tablet Formulations of Pirfenidone in the Real-Life French RaDiCo-ILD Cohort. Adv Ther. 2022 Jan;39(1):405-420. doi: 10.1007/s12325-021-01961-x. Epub 2021 Nov 10.