Airway-centered interstitial fibrosis is a type of interstitial lung disease first proposed in 2004 2. It is still unclear (c.2015) whether it represents a variant of preexisting interstitial lung disease (such as hypersensitivity pneumonitis or organizing pneumonia) or constitutes a separate entity 1,3.
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Terminology
This condition may also be referred to radiologically as bronchiolocentric interstitial pneumonia, but airway-centered interstitial fibrosis is the preferred terminology 1.
Epidemiology
Patients are usually around 50–60 years of age, with a slight female predominance 1,3.
Pathology
Etiology
A history of exposure to inhaled agents, either organic or inorganic, seems to be present in most patients 3.
Microscopic appearance
The main histologic findings are predominant bronchiolocentric interstitial fibrosis with mild inflammatory cell infiltration and peribronchiolar metaplasia 1,3. Granulomas are usually absent or incidental.
Associations
Common associations include 1:
Radiographic features
CT
Imaging findings consist of 3:
diffuse reticulation around the airways
areas of ground glass opacity
peribronchial interstitial thickening
Subpleural involvement, honeycombing and consolidation are usually absent 3.
Treatment and prognosis
Potential treatments include antigen avoidance, immunosuppressive therapy (corticosteroids) and antireflux measures 1,3.
It is thought to have better survival when compared with idiopathic pulmonary fibrosis 1.
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