Fibrose pulmonaire idiopathique

54 recommandations ont été formulées, .. portant sur des aspects multiples de la maladie : épidémiologie, modalités diagnostiques, critères de qualité et interprétation du scanner thoracique, indication et réalisation de la biopsie pulmonaire, bilan étiologique, modalités et indications de l’enquête familiale et de l’analyse génétique, évaluation du retentissement fonctionnel et du pronostic, indication et emploi des médicaments antifibrosants, transplantation pulmonaire

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

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

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

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.