Maladie pulmonaire interstitielle (chronique)

Code/s CIM-10 : J84(idiopath.), J60-J70(agents ext)
Auteur : Dr. Carmelo Lafuente
Dernière mise à jour : 30/03/2026

 Une longue liste de maladies peuvent produire une atteinte de l'interstitium pulmonaire et évoluer +/-lentement vers la fibrose pulmonaire. Il f

Une longue liste de maladies peuvent produire une atteinte de l'interstitium pulmonaire et évoluer +/-lentement vers la fibrose pulmonaire.

Il faut distinguer les maladies interstitielles qui ont une cause externe ou sont associées à des maladies systémiques, et celles idiopathiques, classifiées en plusieurs types : fibrose pulmonaire idiopathique, pneumopathie interstitielle non-spécifique (PINS), PNP organisée cryptogénique, bronchiolite respiratoire avec PNP interstitielle, PNP interstitielle desquamative et autres, rares.

Le pronostique et les possibilités de traitement dependent de type spécifique=> un bon travail diagnostique (histoire détaillée !) est essentiel et un avis spécialisé recommandé+++.

Causes :

Externes : tabac, médicaments++, poussières (fibres synthétiques, silice, métaux, bois..), vapeurs chimiques, poussières organiques (pollen, plumes..)→alvéolite allergique.
- Infections : mycobactéries, pneumocystis...
- Cancer : adénoK alvéolaire, lymphome.
- Sarcoïdose. Maladies auto-immunes : vascularites, collagénoses...
- Idiopathiques, enfin.

Traitements

Liste des traitements : 1ème ligne
  • Aucun.e traitement renseigné.e pour cette maladie, pour l'instant.

Examens diagnostiques

  • Aucun.e examen renseigné.e pour cette maladie, pour l'instant.

Scores, Listes utiles

  • Aucun.e score renseigné.e pour cette maladie, pour l'instant.

Références

  • Dempsey 2010. Revue narrative : How to investigate a patient with suspected interstitial lung disease. BMJ
    Some commonly prescribed drugs, environmental dusts, smoking, and underlying connective tissue diseases can cause interstitial lung disease. Detailed pulmonary function tests and high resolution computed tomography are usually done ... and some patients may need a lung biopsy. Refer to a chest physician with an interest in interstitial lung diseases for diagnosis and management by a multi-disciplinary team
  • Recos Europe ERS/EULAR 2025. Recommandations de pratique clinique : ERS/EULAR clinical practice guidelines for connective tissue diseases associated interstitial lung disease. Ann Rheum Dis
    ... recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). .. Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice
  • Recos International 2018. Recommandations de pratique clinique : Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care
    The guideline panel updated the diagnostic criteria for IPF. Previously defined patterns of usual interstitial pneumonia (UIP) were refined to patterns of UIP, probable UIP, indeterminate for UIP, and alternate diagnosis. For patients with newly detected interstitial lung disease (ILD) who have a high-resolution computed tomography scan pattern of probable or indeterminate for UIP, or an alternative diagnosis, conditional recommendations were made for performing BAL and surgical lung biopsy; In contrast, fo
  • Recos Internationales IPF 2022. Recommandations de pratique clinique : Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med
    1) Update of idiopathic pulmonary fibrosis (IPF): A conditional recommendation was made to regard transbronchial lung cryobiopsy as an acceptable alternative ... No recommendation was made for or against genomic classifier testing. Conditional recommendations were made against antacid medication and antireflux surgery for the treatment of IPF. 2) Progressive pulmonary fibrosis: PPF was defined as at least two of three criteria (worsening symptoms, radiological progression, and physiological progression) occ
  • Recos USA ATS 2025. Recommandations de pratique clinique : Approach to the Evaluation and Management of Interstitial Lung Abnormalities: An Official American Thoracic Society Clinical Statement. Am J Respir Crit Care Med
    interstitial lung abnormalities (ILAs) [are] defined as nondependent bilateral parenchymal abnormalities on computed tomography, including ground-glass opacities or reticulations, lung distortion, traction bronchiectasis, and/or honeycombing involving ≥5% of a lung zone. The updated definition removes the prior exclusion of high-risk populations. Interstitial lung disease (ILD) is distinguished from ILAs by symptoms (dyspnea/cough) .., abnormal or declining lung function, fibrotic (honeycombing and/or retic
  • Wijsenbeek 2020. Revue narrative : Spectrum of Fibrotic Lung Diseases. N Engl J Med
    This review covers fibrotic pulmonary diseases. Although idiopathic pulmonary fibrosis is very common, the review focuses mainly on fibrotic diseases other than idiopathic pulmonary fibrosis. The biology, clinical presentation, and treatment of the more common disorders are discussed.