Respiratory bronchiolitis-interstitial lung disease (RB-ILD)

Case contributed by Domenico Nicoletti
Diagnosis almost certain

Presentation

Dyspnea and chronic cough in heavy smoker of forty cigarettes a day for thirty years.

Patient Data

Age: 55 years
Gender: Male

Ground glass patchy areas with some concomitant centrilobular nodules and peripheral centrilobular emphysema are seen in both lungs, prevalent in the upper lobes.

Bronchoalveolar lavage (BAL)

Increased total number of cells with accumulation of macrophages that contain brownish pigment in the respiratory bronchioles and alveoli with modest increase in neutrophils and mild fibrosis, compatible with respiratory bronchiolitis.

Pulmonary function tests

Mixed, predominantly obstructive, combined with hyperinflation, associated with a moderate reduction in DLCO.

Case Discussion

RB-ILD is histologically characterized by an intraluminal and intra-airspace accumulation of pigmented macrophages associated with peribronchiolar inflammation and fibrosis. RB-ILD is nearly always associated with cigarette smoking, and strongly associated with centrilobular emphysema. Sometimes there are patchy areas of hypoattenuation suggesting air trapping due to small airway disease.

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