[Idiopathic pulmonary fibrosis]

Jean-François Cordier
La Revue du Praticien 2007 December 31, 57 (20): 2227-34
Idiopathic pulmonary fibrosis (IPF) is currently defined by a set of histopathological-radiological-clinical criteria including an histopathological pattern of usual interstitial pneumonia, reticular opacities with honeycombing at tomodensitometry, progressive exercise dyspnea in a patient older than 60 years with fine crackles at lung bases at auscultation. The incidence of IPF is increasing. Risk factors for the disease have been identified (smoking, exposure to various dusts, genetic polymorphism) but the true cause of FPI remains unknown. Progression to death within 2-4 years may be accelerated by acute exacerbation which may cause death. There is no efficient treatment for IPF. Corticosteroids and immunosuppressors are of no benefit. Therapeutic trials are on the way.

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