PMC6282426_01
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.
ZECR_A_1552065_F0001_B.jpg
PMC6282426_01_ZECR_A_1552065_F0001_B.jpg
F0001
Repetitive chest x-rays (CXR) showed a reticular pattern and bilateral infiltrates (Figure 1).
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.
ZECR_A_1552065_F0002_B.jpg
PMC6282426_01_ZECR_A_1552065_F0002_B.jpg
F0002
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).
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.
ZECR_A_1552065_F0003_PB.jpg
PMC6282426_01_ZECR_A_1552065_F0003_PB.jpg
F0003
Supplemental transbronchial biopsies (TBB) performed during bronchoscopy revealed intraalveolar accumulation of a proteinaceous material, which was consistent with the diagnosis PAP (Figure 3).