Search
respiratory bronchiolitis associated interstitial lung disease; desquamative interstitial pneumonia (RBILD)
Etiology: idiopathic
Epidemiology:
1) smokers 40-60 years of age
2) relatively rare
Pathology:
1) inflammation of respiratory bronchioles, alveolar ducts, & alveoli
2) mild interstitial fibrosis
3) pigmented alveolar macrophages within distal airways, airsacs & alveoli
4) metaplasia of respiratory epithelium
Clinical manifestations:
1) cough
2) dyspnea
3) may be incidental finding in an otherwise asymptomatic active skoker
Special laboratory:
- pulmonary function testing:
a) obstructive or mixed obstructive-restrictive pattern
b) diminished DLCO
Radiology:
1) chest X-ray
a) diffusely prominent vascular/interstitial markings
b) 'dirty lungs'
2) high resolution computed tomography (CT) of thorax
a) diffuse ground-glass opacities with little fibrosis
b) may show air-trapping or cysts
c) mid to upper lung centrilobular micronodules [1]
d) may be normal
Management:
1) smoking cessation
2) corticosteroids generally results in improvement
3) prognosis is good
General
bronchiolitis
interstitial pneumonia
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 17, 18.
American College of Physicians, Philadelphia 1998, 2015, 2018.