[Idiopathic pulmonary fibrosis].
Idiopathic pulmonary fibrosis is the most common of the idiopathic interstitial pneumonias. It occurs more often in men than in women and in those older than 65 years than in younger people. It is strongly associated with smoking. Diffuse crackles at auscultation and computed tomographic features of reticulation and honeycombing are major elements of diagnosis. The concept of epithelial-mesenchymal cell transition has recently received much attention; this transition appears to play a greater role in the pathogenesis than inflammation. The recently identified syndrome of combined pulmonary fibrosis and emphysema is characterized by relatively preserved lung volumes and flows contrasting with severely impaired carbon monoxide transfer, major exercise hypoxemia, and an elevated prevalence of pulmonary hypertension. Disease exacerbations contribute to deterioration and to death. There is no effective treatment for this disease. Median survival is about 2-3 years after diagnosis.
Cordier
Jean-François
JF
Service de pneumologie, Centre de référence des maladies rares pulmonaires, Hôpital cardio-vasculaire et pneumologique Louis Pradel, F-69677 Lyon (Bron) Cedex, France. jean-francois.cordier@chu-lyon.fr
D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D018893:Bronchoalveolar Lavage; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D054990:Idiopathic Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008297:Male; D011656:Pulmonary Emphysema / Q000473:pathology; D011859:Radiography