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Thorax 66:624-629 doi:10.1136/thx.2009.134106
  • Review

Airway remodelling and its relationship to inflammation in cystic fibrosis

  1. Jane C Davies1,2
  1. 1Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
  2. 2Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, UK
  3. 3Division of Paediatric Respiratory Medicine, University Children's Hospital, Bern, Switzerland
  1. Correspondence to Nicolas Regamey, Division of Paediatric Respiratory Medicine, University Children's Hospital, 3010 Bern, Switzerland; nicolas.regamey{at}insel.ch
  • Received 28 December 2009
  • Accepted 9 June 2010
  • Published Online First 1 October 2010

Abstract

Pulmonary disease is the most important cause of morbidity and mortality in cystic fibrosis (CF). Most patients with CF die from respiratory failure with extensive airway destruction. Airway remodelling, defined as structural airway wall changes, begins early in life in CF but the sequence of remodelling events in the disease process is poorly understood. Airway remodelling in CF has traditionally been thought to be solely the consequence of repeated cycles of inflammation and infection. However, new evidence obtained from developmental, physiological and histopathological studies suggests that there might instead be multiple mechanisms leading to airway remodelling in CF including (1) changes related to infection and inflammation; (2) changes specific to CF as a result of CF transmembrane conductance regulator (CFTR) dysfunction in the airway wall, independent of infection and inflammation; and (3) protective responses to (1) and/or (2). Recent advances in bronchoscopic techniques have allowed airway mucosal (endobronchial) biopsies to be taken in children and even infants. Endobronchial biopsy studies may provide insight into the role and relative contribution of the different mechanisms of airway remodelling in CF, with the main limitation that they assess only changes in proximal large airways and not in peripheral small airways from where CF disease is thought to originate. Findings from biopsy studies could encourage the development of novel therapeutic strategies targeting structural changes in addition to infection and inflammation.

Footnotes

  • Funding NR is the recipient of a European Respiratory Society Fellowship (Nr. 64).

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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