Pulmonary alveolar proteinosis

Case contributed by Bruno Di Muzio
Diagnosis certain

Presentation

Progressive dyspnoea and dry cough.

Patient Data

Age: 70 years
Gender: Female

Chest radiograph

x-ray

Bilateral widespread and symmetric ground glass opacity, with relative sparing of the apices. No pleural effusions. The cardiomediastinal contour is normal. 

CT Chest

ct

Widespread symmetrical ground glass opacities associated with inter- and intra-lobular septal thickening (crazy paving pattern). There is subpleural sparing. Airways are normal, no pleural effusion. The mediastinal structures are normal, no lymphadenopathy. 

The patient had complete bronchial/lung lavage on bronchoscopy. 

Macroscopy: Bronchial washings - lightly, blood-stained fluid.

Microscopy:  Smears show larger, acute inflammatory cells, alveolar macrophage, and background of PAS-positive proteinaceous material. Findings are in keeping with alveolar proteinosis.

Case Discussion

The diagnosis was confirmed on bronchoscopic washings as pulmonary alveolar proteinosis (PAP), with further clinical improvement after the bronchoalveolar lavage and corticosteroid treatment.

PAP typically presents with slowly progressive dyspnoea over months or years and this history helps to distinguish this condition from other causes of crazy paving.

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