Regulatory requalification en C20-58

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.

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.

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

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.