PMC6175766_01
Chest computed tomography (CT) shows emphysema in bilateral upper lobes (A) and reticulation with honeycombing in bilateral lower lobes (B), suggestive of combined pulmonary fibrosis with emphysema (CPFE). In addition, high-resolution CT (HRCT) reveals the extent of ground-glass opacities (GGO) around emphysema and fibrotic lesion, multiple nodules, and thickening of bronchovascular bundles (C).
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Chest computed tomography (CT) showed emphysema in bilateral upper lobes, and reticulation with honeycombing in bilateral lower lobes, suggestive of combined pulmonary fibrosis with emphysema (CPFE) (Fig. 1A and B). In addition, high-resolution CT (HRCT) revealed the extent of ground-glass opacities (GGO) around emphysema and interstitial fibrotic change, multiple nodules and thickening of bronchovascular bundles (Fig. 1C).
Histopathological findings of video-assisted thoracoscopic lung biopsy specimen from the right S3 and S5. The specimens showed patchy distribution and involvement of the subpleural and paraseptal fibrosis with airspace enlargement and honeycombing (A: x 4, hematoxylin-eosin [HE] stain). There is a dense lymphoplasmacytes infiltration with lymphoid aggregates in the alveolar septa and bronchovascular bundles (B: x 40, HE stain). Plasma cell infiltration is prominent (C: x 200, HE stain), and the immunohistochemical staining with IgG (D: x 200) and IgG4 (E: x 200) shows an IgG4/IgG-positive cell ratio of approximately 65%.
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Histopathological examination revealed a patchy distribution and involvement of the subpleural and paraseptal fibrosis with airspace enlargement and honeycombing (Fig. 2A), suggesting a usual interstitial pneumonia (UIP) pattern. However, intriguingly, there was marked lymphoplasmacytes infiltration with lymphoid aggregates in the alveolar septa and bronchovascular bundles, especially in dense fibrotic lesions (Fig. 2B and C). The immunohistochemical staining revealed that most of the infiltration plasma cells were positive for IgG and IgG4 (Fig. 2D and E).
Chest computed tomography (CT) after 6 months of corticosteroid therapy shows significantly improvement of ground-glass opacities (GGO) and/or consolidation around emphysema and fibrotic change in upper lobes (A) and in lower lobes (B). High-resolution CT (HRCT) reveals that the extent of GGO disappearance, loss of multiple nodules, and the decrease in the thickening of bronchovascular bundles (C).
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The improvement was characterized by the disappearance of GGO around emphysema and interstitial fibrotic changes, loss of multiple nodules, and decreased thickening of bronchovascular bundles (Fig. 3A-C).