Pathogenesis, current treatments and future directions for idiopathic pulmonary fibrosis

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HighlightsTherapy with prednisone/azathioprine/N-acetylcysteine increases mortality in IPF.Several molecular pathways contribute to the pathogenesis of IPF.Heterogeneity in IPF emphasizes the need for patient specific treatments.Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease (ILD) of unknown origin characterized by epithelial cell dysfunctions, accumulation of fibroblasts and myofibroblasts and relentless deposition of extracellular matrix (ECM). Improved diagnostic accuracy and better trial design have provided important insights from recent clinical trials. Perhaps the most important insight was the realization that ‘standard therapy’ was actually harmful! This review summarizes the current understanding of the cell types that are altered in IPF and the pathogenic mechanisms that have been identified. It also reviews recent clinical trial results and interpretations. Finally, we highlight attractive biologic targets and therapies in development with recommendations for future therapeutic avenues.

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