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Thorax 62:638-649 doi:10.1136/thx.2004.031005
  • Review series

Challenges in pulmonary fibrosis · 2 : Bronchiolocentric fibrosis

  1. Jean-François Cordier
  1. Correspondence to:
    Professor Jean-François Cordier
    Claude Bernard University and Department of Respiratory Medicine, Reference Center for Orphan Pulmonary Diseases, Louis Pradel University Hospital, 69677 Lyon (Bron), France; jean-francois.cordier{at}chu-lyon.fr
  • Received 30 January 2006
  • Accepted 18 July 2006

Abstract

Bronchiolocentric fibrosis is essentially represented by the pathological pattern of constrictive fibrotic bronchiolitis obliterans. The corresponding clinical condition (obliterative bronchiolitis) is characterised by dyspnoea, airflow obstruction at lung function testing and air trapping with characteristic mosaic features on expiratory high resolution CT scans. Bronchiolitis obliterans may result from many causes including acute diffuse bronchiolar damage after inhalation of toxic gases or fumes, alloimmune chronic processes after lung or haematopoietic stem cell transplantation, or connective tissue disease (especially rheumatoid arthritis). Airway-centred interstitial fibrosis and bronchiolar metaplasia are other features of bronchiolocentric fibrosis.

Footnotes

  • Competing interests: None.