Abstract
Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic,progressive fibrosing interstitial pneumonia of unknown cause,characterized by a histological pattern of usual interstitial pneumonia.The clinical course of IPF is usually chronic,but some patients may experience episodes of acute respiratory worsening.These episodes of idiopathic acute deterioration have been termed as acute exacerbations of IPF (AE-IPF).AE-IPF may be less predictable,associated with a high mortality rate and no effective therapy.This article reviews the incidence,risk factors,pathophysiology,pathogenesis,diagnosis and treatment progress in AE-IPF. Key words: Idiopathic pulmonary fibrosis ; Acute exacerbation; Biomarkers ; Treatment
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