The Processes and Mechanisms of Cardiac and Pulmonary Fibrosis

Lucy A. Murtha, Michael J. Schuliga, Nishani S. Mabotuwana, Sean A. Hardy, David W. Waters, Janette K. Burgess, Darryl A. Knight, Andrew J. Boyle*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

59 Citations (Scopus)
283 Downloads (Pure)

Abstract

Fibrosis is the formation of fibrous connective tissue in response to injury. It is characterized by the accumulation of extracellular matrix components, particularly collagen, at the site of injury. Fibrosis is an adaptive response that is a vital component of wound healing and tissue repair. However, its continued activation is highly detrimental and a common final pathway of numerous disease states including cardiovascular and respiratory disease. Worldwide, fibrotic diseases cause over 800,000 deaths per year, accounting for similar to 45% of total deaths. With an aging population, the incidence of fibrotic disease and subsequently the number of fibrosis-related deaths will rise further. Although, fibrosis is a well-recognized cause of morbidity and mortality in a range of disease states, there are currently no viable therapies to reverse the effects of chronic fibrosis. Numerous predisposing factors contribute to the development of fibrosis. Biological aging in particular, interferes with repair of damaged tissue, accelerating the transition to pathological remodeling, rather than a process of resolution and regeneration. When fibrosis progresses in an uncontrolled manner, it results in the irreversible stiffening of the affected tissue, which can lead to organ malfunction and death. Further investigation into the mechanisms of fibrosis is necessary to elucidate novel, much needed, therapeutic targets. Fibrosis of the heart and lung make up a significant proportion of fibrosis-related deaths. It has long been established that the heart and lung are functionally and geographically linked when it comes to health and disease, and thus exploring the processes and mechanisms that contribute to fibrosis of each organ, the focus of this review, may help to highlight potential avenues of therapeutic investigation.

Original languageEnglish
Article number777
Number of pages15
JournalFrontiers in Physiology
Volume8
DOIs
Publication statusPublished - 12-Oct-2017

Keywords

  • Acute respiratory distress syndrome
  • Cardiac fibrosis
  • Heart
  • Heart failure
  • Idiopathic pulmonary hypertension
  • Lung
  • Myocardial infarction
  • Pulmonary fibrosis
  • adult respiratory distress syndrome
  • aging
  • death
  • heart failure
  • heart infarction
  • heart muscle fibrosis
  • human
  • lung fibrosis
  • major clinical study
  • morbidity
  • mortality
  • pulmonary hypertension
  • regeneration
  • biological product

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