Constrictive Bronchiolitis Obliterans: The Fibrotic Airway Disorder

Gary R. Epler

Disclosures

Expert Rev Resp Med. 2007;1(1):139-147. 

In This Article

Pathological Description

The term 'constrictive bronchiolitis' (or 'CB') is used for the concentric fibrotic lesion of the bronchiolar submucosa. This lesion is characterized by "a peribronchiolar fibrotic process that surrounds rather than fills the lumen", resulting in extrinsic compression and obliteration of the airway.[1] There is mural thickening by submucosal collagenous fibrosis with progressive concentric narrowing associated with luminal distortion, mucous stasis and chronic inflammation.[2] CB is a late, fibrotic, concentric bronchiolitis that occurs with or without complete obliteration.[3]. Popper wrote that CB was called 'fibrosing bronchiolitis' in the old German pathology literature.[4] The lesion preferentially involves membranous bronchioles and is characterized by fibrosis of the stroma and narrowing of the lumen in a concentric fashion. The muscle layer may be hypertrophic in early lesions, atrophic in late stages and replaced by fibrotic tissue at the end stage. Homma and colleagues noted that constrictive obliterative bronchiolitis is characterized by concentric narrowing or complete obliteration of the airway lumen due to a submucosal lesion occurring in the membranous bronchioles and sparing the distal respiratory bronchioles.[5] Visscher and Myers noted that CB is often patchy and focal, making the diagnosis difficulty from a transbronchial biopsy, and advanced cases may be especially inconspicuous because of lack of active inflammation and disappearance of bronchioles[6].

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