REVIEW PAPER OF IDIOPATHIC PULMONARY FIBROSIS

Idiopathic pulmonary fibrosis is a rapidly progressive illness of unknown cause characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. IPF is triggered by an as yet unidentified alveolar injury that leads to activation of transforming growth factor-? (TGF-?) and alveolar basement membrane disruption. The resulting deposition of excess disrupted matrix by these myofibroblasts leads to the development of IPF. Till today, no drug therapy has clearly been demonstrated to benefit patients with idiopathic pulmonary fibrosis. This review highlights the current understanding of IPF, molecular mechanisms of pathogenesis and some diagnosis and possible clinical treatments of this devastating disease.