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Challenges in pulmonary fibrosis · 2 : Bronchiolocentric fibrosis

Jean-François Cordier

Correspondence to:
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

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.

Abbreviations: BOOP, bronchiolitis obliterans with organising pneumonia; BOS, bronchiolitis obliterans syndrome; COPD, chronic obstructive pulmonary disease; FEF_25–75, mid expiratory flow rate between 25% and 75% of FVC; FEV₁, forced expiratory volume in 1 s; FVC, forced vital capacity; GVHD, graft-versus-host disease; HLA, human leucocyte antigen; HRCT, high resolution computed tomography; HSC, haematopoietic stem cell; RV, residual volume; TLC, total lung capacity; TNF, tumour necrosis factor

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