Pulmonary alveolar proteinosis with upper-lobe predominance in a non-smoking female

In this report, we describe an unusual manifestation of pulmonary alveolar proteinosis (PAP). The patient is a 43-year-old non-smoking female without underlying hematologic or auto-immune disorder. Her initial presentation included non-specific respiratory symptoms (exertional dyspnoea and cough), an unremarkable physical examination, a mild elevation in her serum level of lactate dehydrogenase, a mild impairment in the diffusion capacity for carbon monoxide but a normal spirometry, and multiple ground-glass opacities with a “crazy-paving” pattern predominantly in upper lung zones on her chest radiographic images. PAP was diagnosed histologically. PAP commonly occurs in males with smoking history, and tends to affect the lung parenchyma diffusely or, as in auto-immune PAP, lower lobes predominantly. Upper-lobe predominant PAP, particularly in a non-smoking female, is rare. This report would add PAP to the list of differential diagnosis for upper-lung ground-glass opacities. A review on the relevant literature is also included in the discussion.