Idiopathic Interstitial Pneumopathy/Pneumopathy Interstitial Diffuse, Paediatric and Adult Patients
Conditions
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.
Interventions
None listed
Sponsors
Institut National de la Santé Et de la Recherche Médicale, France
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| The phenotypic description idiopathic lung disease | Up to 10 years | 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) |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Identify gene factors involved in disease initiation and progression | Up to 10 years | study genes : SFTPA1, SFTPA2, SFTPB, SFTPC, ABCA3, NKX-2.1, TERT, TERC, RTEL1, PARN, DKC1, TINF2, COPA, MARS, CSF2RA, CSF2RB, SERPINA1, FLCN |
| Investigate the extent to which environmental and co-morbidity factors may influence disease severity and outcome | Up to 10 years | 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 |
| Identify and validate biomarkers for disease diagnosis and progression | Up to 10 years | 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) |
Countries
France
Outcome results
None listed