PMC5313428_01
Chest X-ray film showing bilateral GGO in both lower lung fields.
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Chest X-ray showed bilateral ground-grass opacity (GGO) in both of the lower lung fields (Fig. 1).
Chest HRCT showing peribronchovascular predominant reticular abnormalities and GGO. The resection site of VATS is shown at the fence line (a: right S3, b: right S6, c: right S8).
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Chest CT showed bronchovascular bundle-dominant reticular shadows and GGO in the bilateral lung field (Fig. 2, 7a). Lung tissue was obtained by VATS from three parts of the lung (right S3, right S6, and right S8) (Fig. 2).
(a) The histological findings of the VATS specimens from the right upper lobe (S3) showing the patchy distribution of mural incorporation fibrosis (a ), lymphatic follicles (b ), and the partial distribution of subpleural fibrosis ( ). (b) The histological findings of the VATS specimens from the right lower lobe (S8) showing widespread interstitial fibrosis with airspace enlargement (Hematoxylin and Eosin staining; original magnification a: x10, b: x12.5).
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In contrast, the primary lesions of right S8 were fibrotic non-specific interstitial pneumonia (f-NSIP)-like lesions (Fig. 3, 4).
Higher-power images of VATS specimens. (a) Mural incorporation fibrosis of the alveolar wall associated with intra-alveolar exudate in the right S3 section. (b) Slight infiltration of lymphocytes (L ) and eosinophils (E ) in the alveolar wall in the right S3 section. (c) Subpleural fibrosis of the right S3 section showing dense fibrosis, the collapse of alveolar walls, and a fibroblastic focus ( ). (d) Widespread interstitial fibrosis with airspace enlargement and bronchiolar metaplasia in the right lower lobe (S8) section. (e) The patchy involvement of mural incorporation fibrosis in the right S8 section. (f) Subpleural and paraseptal fibrosis in the right S8 section (Hematoxylin and Eosin staining; original magnification a, d, e: x100, b: x200, c, f: x40).
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In contrast, the primary lesions of right S8 were fibrotic non-specific interstitial pneumonia (f-NSIP)-like lesions (Fig. 3, 4).
The histological findings of TBLB specimens showing peribronchiolar metaplasia (a ) and slight mononuclear cell infiltration to the bronchiolar wall (b ) (Hematoxylin and Eosin staining; original magnificationx100).
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TBLB specimens and cytology of the bronchoalveolar lavage fluid (BALF) showed no leukemic cells (Fig. 5).
Clinical course.
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The GGO, KL-6, and SP-D improved after combined therapy with pirfenidone and erythromycin (Figure 6, 7).
The chest HRCT findings. (a) Before being treated, basal and bronchovascular bundle-dominant reticular shadows and GGO were shown. (b) Two months after treatment with pirfenidone 1,800 mg/day, the reticular shadows and GGO slightly improved. (c) Three months after a dose reduction to 1,200 mg/day, the basal and bronchovascular bundle-dominant reticular shadows and GGO had deteriorated. (d) Nine months after treatment with pirfenidone 1,200 mg/day and erythromycin 400 mg/day, the reticular shadows and GGO continued to improve.
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Chest CT showed bronchovascular bundle-dominant reticular shadows and GGO in the bilateral lung field (Fig. 2, 7a). The CT findings and the dyspnea on exertion subsequently improved (Fig. 7b); however, the KL-6 and SP-D increased slightly (Fig. 6, clinical course). The symptom of dyspnea on exertion, the percutaneous oxygen saturation in room air, and the findings on the pulmonary function did not progress (Table 2); however, the CT findings of bronchovascular bundle-dominant reticular shadows and GGO had deteriorated 6 months after the initiation of the pirfenidone treatment (Fig. 7c). The GGO, KL-6, and SP-D improved after combined therapy with pirfenidone and erythromycin (Figure 6, 7).