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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.
Condition or disease
Idiopathic Interstitial Pneumopathy/Pneumopathy Interstitial DiffusePaediatric and Adult Patients
The French RaDiCo-IIP/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 Disesa (ILD) from the reference and competence centers.
Investigate the extent to which environmental and co-morbidity factors may influence disease severity and outcome [ Time Frame: 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 [ Time Frame: 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)
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Layout table for eligibility information
Ages Eligible for Study:
Child, Adult, Older Adult
Sexes Eligible for Study:
Accepts Healthy Volunteers:
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.
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 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