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• W2012030646 abstract "Idiopathic pulmonary fibrosis (IPF) is a highly lethal disor­ der associated with an extremely poor prognosis and poor survival in most Higher mortality rates attributable to pulmonary fibrosis are being increasingly recognized, 1 the median duration of survival being approximately 4 years. This progressive and generally fatal condition is character­ ized by inflammation, fibrosis, and altered connective tissue in the pulmonary parenchyma. Although increasing knowl­ edge of cytokine biologic features and of the complex cytokine-cytokine-cell matrix interactions has shed useful light on the genesis of pulmonary fibrosis.v' IPF continues to pose major clinical challenges and to frustrate physicians and patients because an effective therapeutic regimen is yet to be determined. Treatment Controversy.-Once IPF has been diagnosed, decisions must be made about whether to treat it and what therapeutic regimens should be used. These decisions are complicated by the unpredictability of the clinical course in a specific patient and the relatively low response rate to avail­ able therapeutic agents.' Corticosteroids are currently the mainstay of pharmacologic therapy for IPF; however, the data supporting the oral use of long-term maintenance corti­ costeroid therapy for IPF are weak and are generally based on retrospective, uncontrolled trials. The optimal dosage and duration of treatment are unclear, and clinical responses are seen in only 20 to 30% of patients. The response is almost never dramatic, and the treatment with corticoste­ roids is inevitably associated with pronounced adverse ef­ fects that increase the morbidity for the already suffering patient. Despite this awareness, some experts believe that treatment with corticosteroids with or without adjunctive immunosuppressive agents should be given to all patients with the hope that such a therapeutic regimen might decrease the rate of progression of pulmonary fibrosis, at least in a subgroup of Others think that such treatment is potentially harmful and may be generally futile, particularly in elderly patients, and do not universally recommend treat" @default.
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• W2012030646 date "1997-03-01" @default.
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• W2012030646 title "Idiopathic Pulmonary Fibrosis: A Need for Treatment With Drugs Other Than Corticosteroids—a Role for Antifibrotic Agents?" @default.
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• W2012030646 doi "https://doi.org/10.4065/72.3.285" @default.
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