La Revue du praticien
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Idiopathic interstitial pneumonias represent approximately 30% of all interstitial lung diseases. The new classification of idiopathic interstitial pneumonias published in 2013 distinguishes 6 major entities, including chronic fibrosing forms (idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia), acute/subacute forms (cryptogenic organizing pneumonia and acute interstitial pneumonia) and smoking-related disorders (respiratory bronchiolitis interstitial lung disease and desquamative interstitial pneumonia). Pleuroparenchymal fibroelastosis is individualized as a new rare clinco-pathologic entity. For cases not fitting any specific clinic- pathological category, a pragmatic classification based on disease behavior is proposed.
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Fibroblasts are key cells in the fibrotic process. Their profibrotic phenotype is influenced by the environment in which they operate, both extracellular matrix and mediators which they are exposed. The pharmacologic modulation of the fibroblast phenotype allows to identify potential therapeutic targets which might interrupt the vicious cycle of pulmonary fibrogenesis.