Respiratory Bronchiolitis-Associated Interstitial Lung Disease (RB-ILD)
Epidemiology:
- Cigarette smokers
(avg 30 pack-years)
- M:F=2:1
- 30s to 40s onset
Common sites:
Gross features:
Histologic features:
- patchy changes
with a bronchiolocentric distribution
- pigmented intraluminal macrophages (smokers’ macrophages) within
first- and second-order respiratory bronchioles, alveolar ducts, and peribronchiolar spaces
- patchy submucosal and peribronchiolar
lymphocytic infiltrate
- mild peribronchiolar fibrosis expanding contiguous
alveolar septa
- emphysema often
present (not severe)
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- spectrum with DIP
- often incidental histologic finding in smokers or ex-smokers
- term “RB-ILD”
reserved for symptomatic patients with abnormal PFTs
and imaging
- improvement with
smoking cessation
References: