Presentation
Progressive dyspnoea and dry cough.
Patient Data
Bilateral widespread and symmetric ground glass opacity, with relative sparing of the apices. No pleural effusions. The cardiomediastinal contour is normal.
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.