Vascular remodelling in the pathogenesis of idiopathic pulmonary fibrosis

S Barratt, A Millar

Research output: Contribution to journalReview article (Academic Journal)peer-review

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and irreversible fibrosing interstitial pneumonia of unknown aetiology that usually leads to respiratory failure and death within 5 years of diagnosis. Alveolar epithelial cell injury, disruption of alveolar capillary membrane integrity and abnormal vascular repair and remodelling have all been proposed as possible pathogenic mechanisms. This review summarizes our current knowledge of the abnormalities in vascular remodelling observed in IPF and highlights several of the cytokines thought to play a pathogenic role, which may ultimately prove to be future therapeutic targets.

Original languageEnglish
Pages (from-to)515-9
Number of pages5
JournalQJM
Volume107
Issue number7
DOIs
Publication statusPublished - Jul 2014

Keywords

  • Angiostatic Proteins/physiology
  • Humans
  • Hypertension, Pulmonary/etiology
  • Idiopathic Pulmonary Fibrosis/etiology
  • Microcirculation/physiology
  • Neovascularization, Pathologic/complications
  • Vascular Remodeling/physiology

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