[Idiopathic pulmonary fibrosis].
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.
Cordier
Jean-François
JF
Service de pneumologie, centre de réf6érence des maladies orphelines pulmonaires, hôpital cardiovasculaire et pneumologique Louis-Pradel, université Lyon-1, université de Lyon, 69677 Lyon, Bron. jean-francois.cordier@chu-lyon.fr
D018893:Bronchoalveolar Lavage; D003952:Diagnostic Imaging; D006801:Humans; D016040:Lung Transplantation; D011658:Pulmonary Fibrosis / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D012129:Respiratory Function Tests