Desquamative Interstitial Pneumonia
Epidemiology:
- Cigarette smokers (virtually all)
- 30s to 40s onset
- M:F=2:1
Common sites:
Gross features:
Histologic features:
- Diffuse, temporally uniform
- large collections of airspace macrophages
(originally thought to be desquamated pneumocytes)
- abundant cytoplasm with dusty brown pigment
(smokers’ macrophages)
- finely granular iron may be seen
- lamellar bodies (surfactant) in some
- thickened alveolar septa
- sparse inflammatory infiltrate
- lymphocytes
- plasma cells
- eosinophils occasional
- lined by plump, cuboidal pneumocytes
- minimal (if any) fibrosis
- emphysema often present
Immunophenotype:
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Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- spectrum with RB-ILD
- insidious onset weeks or months
- good prognosis:
- nonprogressive in the vast majority of cases
- excellent response to steroids and smoking
cessation
References: