• Dominique Valeyre and Geneviève Dion.
• Université Paris Nord, EA2363 et Assistance-publique hôpitaux de Paris, Hôpital Avicenne, F-93009 125, rue de Stalingrad--93009 Bobigny France. dominique.valeyre@avc.aphp.fr
• B Acad Nat Med Paris. 2010 Feb 1;194(2):367-81; discussion 381-2.

AbstractIdiopathic pulmonary fibrosis (IPF) is a severe disease with a median survival time of only 24-36 months. It is characterized by inexorably progressive respiratory failure and by acute exacerbations that are often rapidly fatal. The standard treatment based on steroids and immunosuppressive drugs is no longer recommended Lung transplantation is the only treatment with an impact on survival but it concerns only a minority of patients and must be performed early in the disease process. Patients not eligible for transplantation should be given the opportunity to participate in clinical trials of promising new therapies. Many trials have recently been completed or are currently underway, but few results have been published. In the meantime, supportive treatment (oxygen therapy and rehabilitation), vaccination, and treatment of comorbidities (gastroesophageal reflux, sleep apnea) are recommended.

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