[Idiopathic pulmonary fibrosis].
Idiopathic pulmonary fibrosis is a fibroproliferative disorder characterized histopathologically by heterogenous interstitial fibrosis with fibroblastic foci and honeycomb lung. The diagnosis is based on a pluridisciplinary approach, integrating the clinical evaluation, the chest high-resolution computerized tomography features, and the pathological pattern of usual interstitial pneumonia on video-thoracoscopic lung biopsy when performed. When the tomodensitometric pattern is typical (50% of the cases), the diagnosis may be made without a lung biopsy. No treatment has proven benefit in survival. Treatment with N-acetylcysteine in association with corticosteroids and azathioprine has recently shown a modest functional improvement. Clinical trials are strongly needed in this still uncurable disease with median survival at 3 years of only about 50%.
Cottin
Vincent
V
Service de pneumologie, Centre de référence des maladies orphelines pulmonaires, hôpital Louis Pradel, Hospices civils de Lyon, UMR 754, université Lyon I, Bron. vincent.cottin@chu-lyon.fr
Cordier
Jean-François
JF
D001706:Biopsy; D006801:Humans; D008168:Lung / Q000473:pathology; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000175:diagnosis* / Q000188:drug therapy*