[Idiopathic pulmonary fibrosis].
Idiopathic pulmonary fibrosis is a chronic disorder characterized histopathologically by a pattern of usual interstitial pneumonia, with heterogeneous and mutilating interstitial fibrosis with foci of proliferating fibroblasts, honeycomb lung, and little if any inflammation. The diagnosis is based on a pluridisciplinary analysis of the clinical symptoms, the chest high-resolution computerized tomography features, and pathology on video-thoracoscopic lung biopsy when indicated. In half of the cases, the typical tomodensitometric pattern allows to make a confident diagnosis without a lung biopsy. The median survival is only about 3 years and is presently not improved by any treatment. Treatment with N-acetylcysteine (antioxydant) in association with corticosteroids and azathioprine may slightly reduce the rate of functional worsening. Clinical trials are in progress to improve the treatment of this still incurable disease.
Cottin
Vincent
V
Service de Pneumologie, Centre de Référence des Maladies Orphelines Pulmonaires, Hôpital Louis Pradel, Hospices Civils de Lyon, Université Lyon I, UMR 754 INRA ENVL IFR128, Lyon, France. vincent.cottin@chu-lyon.fr
Cordier
Jean-François
JF
D001706:Biopsy / Q000379:methods; D018893:Bronchoalveolar Lavage; D005347:Fibroblasts / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D012307:Risk Factors; D020775:Thoracic Surgery, Video-Assisted / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods