case_images: PMC6282426_01
case_images: Chest X-ray often reveal bilateral symmetrical opacities centrally in mid and lower lung zones and sometimes reticular markings. Segmental atelectasis may occur due to obstruction. In chronic cases of PAP focal fibrosis may occur.
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case_images: Repetitive chest x-rays (CXR) showed a reticular pattern and bilateral infiltrates (Figure 1).
case_images: A. Transversal and B.coronal HRCT image during inspiration, where PAP presents by the characteristic crazy paving pattern due to intraalveolar and interlobular interstitial accumulation of surfactant. It is distinctive by the combination of ground glass opacity and sharply delineated polygonal shaped secondary lobuli with septal thickening, giving rise to a cobblestone like pattern.
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case_images: High-resolution computed tomography (HRCT) of the thorax showed interlobular septal thickening with diffuse as well as interlobular ground glass opacities (GGOs) with fluctuating consolidation presenting in a 'crazy paving' pattern (Figure 2).
case_images: Photomicrographs of the transbronchial lung biopsy showed massive filling of alveolar spaces with granular material. The alveolar septae were focally thickened, but otherwise the structure was intact. There were no signs of inflammation or malignancy.
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case_images: Supplemental transbronchial biopsies (TBB) performed during bronchoscopy revealed intraalveolar accumulation of a proteinaceous material, which was consistent with the diagnosis PAP (Figure 3).