Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic,progressive,and fatal fibrotic lung disease of unknown etiology. Clinical trials suggest that pirfenidone, N-acetylcysteine and perhaps anticoagulants,may have some beneficial effects. However,placebo-controlled trials are necessary for confirmation.Corticosteroid with immunosuppression is not proven to have benefits.Lung transplantation has been shown to improve survival in selected IPF patients.Comorbidities accompanying IPF such as gastroesophageal reflux and pulmonary arterial hypertension should be managed appropriately. IPF patients are strongly encouraged to enroll in ongoing clinical trials for this devastating disease. Key words: Idiopathic pulmonary fibrosis; Treatment; Lung transplantation
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