Idiopathic Interstitial Pneumonia: From Infancy to Elderly FRESH-PEF73380-en Sonia GUEGUEN Idiopathic Interstitial Pneumonia: From Infancy to Elderly RaDiCo-PID FRESH-PEF73380-en 73380 FRESH-PEF73380 Vincent COTTIN INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE (INSERM) Epidemiology France Portal (PEF) AGENCE NATIONALE DE LA RECHERCHE (ANR) null Vincent;COTTIN Paediatrics Pulmonary medicine Respiratory diseases principally affecting the lung interstitium Environmental determinants: Climate Socio-demographic and economic determinants Biological determinants: Genetic predisposition Behavioral determinants Environmental determinants Healthcare system determinants: Use of care Socio-demographic and economic determinants: Employment Biological determinants Healthcare system determinants Primary Objective The main objective is to describe the phenotypic features of the paediatric and adult patients with 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. Secondary Objectives The secondary objectives are to: • Identify gene factors involved in disease initiation and progression, • Investigate the extent to which environmental and co-morbidity factors may influence disease severity and outcome • Identify and validate biomarkers for disease diagnosis and progression Exploratory objectives • Production of improved strategies for patient recruitment and enrolment into clinical trials • Development of novel strategy for patient follow-up • Development of novel diagnostic approaches • Evaluation of effect on natural history of disease, and tolerance of therapy, in a large population in real life • Development of novel therapeutic approaches Information Technology Objectives • Develop and diffuse an electronic tool of data collection from various sources linked to a database integrating a system of management and follow-up of data-management allowing collection of data for IIP/PID paediatric and adult patients. • Include data generated by patients and, where relevant, their parents and/or carers. null France Individuals Patients population Male Female Infant, Newborn (birth to 28 days) Infant (28 days to 2 years) Child, Preschool (2 to 5 years) Child (6 to 12 years) Young Adult (19 to 24 years) Adult (25 to 44 years) Middle Aged (45 to 64 years) Aged (65 to 79 years) Aged, 80 and over (80 years and more) Patient with a diagnosis of IIP/PID IIP/PID diagnosis is established on presenting history, clinical, radiological and functional and if available pathological findings. Inclusion criteria include: 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” Patients with diffuse parenchymal lung diseasescaused by drug toxicity, immunodeficiency, proliferative disorders including histiocytosis, and metabolic disordersPatients (parents/guardians for paediatric patient) not able to approve/understand the protocol Clinical data Participant-reported health data Biological data HPO ICD10 Snomed CT Orpha Codes and ORDO Drug dictionary (DCIs) Continuous data management; Data Management Plan and Data Validation Plan. Native controls and Query system Health event/morbidity Health event/mortality Health care consumption and services Quality of life/health perception Others Other Through organizations (health services or institutions schools businesses etc.) [1000-10000[ individuals Converting or copying information into a structured record Medico-administrative database Observational Study Observational Study Morbidity registry 2550 Unknown Restricted access Access requests to RaDiCo-PID data (rough / structured), biocollections or to analytic reports will be examined by the scientific committee following submission of a Specific Research Project (SRP) synopsis, as defined in the Resource Access Charter. Must be sent to pid@radico.fr Access on specific project only