• Vincent Cottin.
• Service de pneumologie, Hôpital Louis Pradel, 69394 Lyon. vincent.cottin@chu-lyon.fr
• Presse Med. 2002 Oct 19; 31 (34): 1613-23.

AbstractA RECENTLY MODIFIED CONCEPT: Idiopathic pulmonary fibrosis (IPF) is characterized by dyspnea on exertion, diffuse radiological infiltrates and alterations in respiratory function. The approach to IPF has recently changed with a more precise definition of the histological diagnostic criteria (hence excluding other disorders such as non-specific interstitial pneumonia), and with the hypothesis that fibro-proliferation and abnormalities in epithelial repair may have a greater physiopathological role than inflammation. DEBATABLE RESULTS FOR CORTICOSTEROIDS: To date, no treatment has demonstrated its efficacy in this disorder and few randomised studies are available. Although early observations showed some benefit of corticosteroids, it is now well established that these studies in fact included a proportion of other corticosteroid-sensitive diseases, such as non-specific interstitial pneumonia. In more recent studies, in which the diagnosis of IPF was made more rigorously, no convincing demonstration of the efficacy of corticosteroids or of immunosuppressive treatments (cyclophosphamide, azathioprine) was made. A trial of corticosteroid therapy for a period of 3 to 6 months (possibly combined with immunosuppressors) is still recommended in the absence of contraindications, but with rigorous and objective assessment of the efficacy, and careful monitoring of the side effects. TREATMENTS UNDER STUDY: Treatments aimed at limiting fibrogenesis have also been proposed. However, clinical studies have not confirmed the initial results obtained with colchicine. Nevertheless, encouraging results have been obtained with other "anti-fibrosing" agents (such as pirfenidone) or immunomodulators (interferon-gamma-1b); such treatment should be further evaluated by larger, randomised, controlled trials in order to know whether these results are applicable to a less selected population. BETWEEN SYMPTOMATIC CARE AND TRANSPLANTATION: In the absence of effective treatment, the management of IPF, the diagnosis of which has been confirmed by rigorous criteria (ideally lung biopsy) is primarily symptomatic. Young patients should be assessed with a view of pulmonary transplantation.

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