Respiratory bronchiolitis interstitial lung disease (RB-ILD): probable
Presentation
Shortness of breath over many months in a heavy smoker. No fevers and no sputum. No evidence of aspiration.
Patient Data
Chest X-ray
Multiple small nodules are seen in both lungs.
The mediastinum is normal, no pleural space abnormality present.
The heart size and shape is normal.
There are no changes of pulmonary edema.
Diffuse centrilobular nodules in both lungs. There is no alveolar or interstitial nor abnormality.
Normal heart size and shape.
These appearances could be due to sarcoid, SAHP, RB-ILD, or pulmonary Langerhans cell histiocytosis. A diffuse infectious bronchiolitis is less likely.
Case Discussion
The patient is a lifelong heavy smoker. There is no suggestion in her history of exposure or evidence of a hypersensitivity pneumonitis.
The diffuse nodules could be due to sarcoidosis, hypersensitivity pneumonitis, respiratory bronchiolitis interstitial lung disease, or even pulmonary Langerhans cell histiocytosis.
Bronchial washings returned a dark stained bronchial lavage, with heavily pigmented macrophages. This is in keeping with a diagnosis of respiratory bronchiolitis interstitial lung disease (RB-ILD).
This is a smoking related interstitial lung disease closely related to respiratory bronchiolitis, but demonstrating more severe histological, imaging and clinical findings.
Desquamative interstitial pneumonitis is also related to smoking, whether DIP and RB-ILD are part of the same disease process or seperate entities is unclear.
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