Advances in acute exacerbation of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is a chronic, progressive and scaring disease with a poor prognosis.Some patients will experience acute exacerbation and rapid deterioration and die.The etiology and pathogenesis of acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) are still unclear.The definition and diagnostic criteria of AE-IPF were updated recently.There is no special effective treatment for AE-IPF till now.This article reviews the research advances in pathogenesis, definition, clinical manifestation, diagnostic criteria and treatment of AE-IPF. Key words: Idiopathic pulmonary fibrosis; Acute exacerbation