4.5 Review Book Chapter

Progress in Understanding and Treating Idiopathic Pulmonary Fibrosis

期刊

ANNUAL REVIEW OF MEDICINE, VOL 70
卷 70, 期 -, 页码 211-224

出版社

ANNUAL REVIEWS
DOI: 10.1146/annurev-med-041317-102715

关键词

interstitial lung disease; familial interstitial pneumonia; genetics; alveolar epithelial cell; fibroblast; cryobiopsy

资金

  1. NHLBI NIH HHS [P01 HL092870, T32 HL094296, K08 HL130595] Funding Source: Medline

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This is a time of substantial progress in the evaluation and care of patients with idiopathic pulmonary fibrosis (IPF). In addition to the approval and widespread availability of the first IPF-specific therapies, there have been improvements in imaging interpretation and lung biopsy methods to enable more expeditious and more accurate diagnosis. Recent advances in identifying genetic factors that underlie susceptibility to IPF and affect prognosis have raised the possibility of personalized therapeutic approaches in the future. Further, evolving work is elucidating novel mechanisms influencing epithelial, mesenchymal, and inflammatory cell responses during the injury-repair process, thus advancing understanding of disease pathogenesis. As analytic approaches mature, the field is now poised to harness the power of rapidly advancing omics technologies to further accelerate progress.

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