PMC4501455_01
Chest computed tomography (CT) scans show paraseptal emphysema in the bilateral upper lobes and reticular lesions with enlarged cysts in the bilateral lower lobes.
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A chest computed tomography (CT) scan showed paraseptal emphysema in the bilateral upper lobes and reticular lesions with enlarged cysts in the bilateral lower lobes (Fig. 1).
A: Lung biopsy specimens from the right S6 obtained by video-assisted thoracoscopic surgery show prominent uniform thickening of alveolar septa by fibrosis (fibrotic nonspecific interstitial pneumonia [f-NSIP] pattern) (EVG stain). B: The alveolar septal thickening mainly consists of collagen deposition with mild inflammatory cell infiltration and lymphoid hyperplasia (HE stain).
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Lung biopsy specimens from the right S6 were obtained by video-assisted thoracoscopic surgery and revealed prominent, uniform thickening of alveolar septa by fibrosis (fibrotic nonspecific interstitial pneumonia [f-NSIP] pattern) (Fig. 2A). The alveolar septal thickening mainly consisted of collagen deposition with mild inflammatory cell infiltration and lymphoid hyperplasia (Fig. 2B).
Lung biopsy specimens from the right S8 showing subpleural and perilobular fibrosis adjacent to relatively normal alveoli (usual interstitial pneumonia [UIP] pattern) (A), as well as fibroblastic foci with dense collagen fibrosis (B) and smooth muscle cell proliferation in areas of dense collagen fibrosis (C).
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Lung biopsy specimens from the right S8 revealed subpleural and perilobular fibrosis adjacent to relatively normal alveoli (usual interstitial pneumonia [UIP] pattern) (Fig. 3A). There were fibroblastic foci with dense collagen fibrosis (Fig. 3B) and smooth muscle cell proliferation in areas of dense collagen fibrosis (Fig. 3C).
Numerous macrophages with a few eosinophils were present in the alveolar lumen (DIP-like lesion) (A), and alveolar wall destruction with enlargement was seen in lung parenchyma (B). Patchy fibrotic lesions were also scattered around the terminal and respiratory bronchioles (C; arrow).
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In addition, numerous macrophages with a few eosinophils were present in the alveolar lumen (DIP-like lesion) (Fig. 4A), and alveolar wall destruction with enlargement was seen in lung parenchyma (Fig. 4B). Patchy fibrotic lesions were also scattered around the terminal and respiratory bronchioles (Fig. 4C).
Clinical course of the patient. MPO-ANCA: myeloperoxidase-antineutrophil cytoplasmic antibody; KL-6: Krebs von den Lungen-6; CCR: creatinine clearance.
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One year later, he died of respiratory failure caused by severe pneumococcal pneumonia (Fig. 5).