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Progress in Understanding and Treating Idiopathic Pulmonary Fibrosis

Authors
  • Kropski, Jonathan A.
  • Blackwell, Timothy S.
Type
Published Article
Journal
Annual Review of Medicine
Publisher
Annual Reviews
Publication Date
Jan 27, 2019
Volume
70
Pages
211–224
Identifiers
DOI: 10.1146/annurev-med-041317-102715
Source
Annual Reviews
Keywords
License
Yellow

Abstract

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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