RaDiCo PID Cohort (RaDiCo-ILD Cohort in English) (RaDiCo-PID)

Idiopathic Interstitial Pneumopathy : Genetic and Environmental Determinants From Infancy to Elderly

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.

Study Overview

• Status: Unknown
• Conditions: Idiopathic Interstitial Pneumopathy/Pneumopathy Interstitial Diffuse; Paediatric and Adult Patients

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.

• Study Type: Observational
• Enrollment (Anticipated): 2500

Contacts and Locations

• Study Contact: Name: Marie Chevereau; Phone Number: + 33 1 71 73 87 94; Email: marie.chevereau@radico.fr
• Study Contact Backup: Name: Sonia Gueguen; Phone Number: +33 6 88 34 54 08; Email: sonia.gueguen@radico.fr

Study Locations

• France
  • Bron, France, 69500
    • Recruiting
    • CHU Lyon - Hôpital Louis Pradel
    • Contact: Vincent Cottin, Pr; Email: vincent.cottin@chu-lyon.fr
  • Paris, France, 75012
    • Recruiting
    • AP-HP - Hôpital Armand Trousseau
    • Contact: Annick Clement, Pr; Email: annick.clement@aphp.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• 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.

Description

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Other

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
children or adults with Idiopathic Lung Disease

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
The phenotypic description idiopathic lung disease	Phenotypic description will be measure demographic data, environmental data, socio-professionnal data, medical history, comorbidities, clinical examination, biological assessment (hematology; biochemistry; hemostasis...), pulmonary biopsy, bronchial-pulmonary imaging; symptom description; respiratory function (arterial blood gas, pulmonary fonction testing, six minute-walk testing, cardiopulmonary exercise testing, polysomnography), treatments, quality of life questionnaire (SF36 and SF10)	Up to 10 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Identify gene factors involved in disease initiation and progression	study genes : SFTPA1, SFTPA2, SFTPB, SFTPC, ABCA3, NKX-2.1, TERT, TERC, RTEL1, PARN, DKC1, TINF2, COPA, MARS, CSF2RA, CSF2RB, SERPINA1, FLCN	Up to 10 years
Investigate the extent to which environmental and co-morbidity factors may influence disease severity and outcome	environnemental data: dwellings, wet areas, dust, smoking, drugs, exposure to materials. Respiratory history, cardiovascular history, endocrinal history, dermatological history, gastroenterology history, gynecology history, haematological history, immune history, neurological history, ophtalmological history, ENT history, psychatric history, Rheumatology history, surgery history	Up to 10 years
Identify and validate biomarkers for disease diagnosis and progression	Identify and validate biomarkers for disease diagnosis and progression with Biological assessment (professionnal data, medical history, comorbidities, clinical examination, biological assessment (hematology; biochemistry; hemostasis...) and respiratory function (arterial blood gas, pulmonary fonction testing, six minute-walk testing, cardiopulmonary exercise testing, polysomnography)	Up to 10 years

Collaborators and Investigators

• Sponsor: Institut National de la Santé Et de la Recherche Médicale, France

Publications and helpful links

General Publications

• Cottin V, Guéguen S, Nunes H, Jouneau S, Crestani B, Bonniaud P, Wemeau L, Israël-Biet D, Reynaud-Gaubert M, Gondouin A, Cadranel J, Marchand-Adam S, Chevereau M, Dufaure-Garé I, Amselem S, Clément 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.

Study record dates

Study Major Dates

• Study Start (Actual): March 28, 2017
• Primary Completion (Anticipated): March 27, 2021
• Study Completion (Anticipated): September 27, 2021

Study Registration Dates

• First Submitted: November 25, 2019
• First Submitted That Met QC Criteria: January 20, 2020
• First Posted (Actual): January 23, 2020

Study Record Updates

• Last Update Posted (Actual): December 14, 2020
• Last Update Submitted That Met QC Criteria: December 10, 2020
• Last Verified: December 1, 2020

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Respiratory Tract Diseases; Lung Diseases
• Other Study ID Numbers: C15-64

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No