PMC8339249_01
Chest radiography revealed consolidation in the right lower lung field at the initial visit.
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Chest radiography showed consolidation in the right lower lung field (Fig. 1).
Pulmonary findings of chest high-resolution computed tomography. (A) At the initial visit, subpleural consolidation with volume reduction in the right lower lobe and localized ground-glass opacity in the left lower lobe were present. (B) Two weeks after she began receiving prednisolone (PSL), consolidation had not improved. (C) Two months after discontinuation of PSL, bilateral consolidation developed. Then, a surgical lung biopsy (SLB) of the right upper lobe was performed (arrow).
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Chest high-resolution CT (HRCT) showed subpleural consolidation with volume reduction in the right lower lobe and localized ground-glass opacity in the left lower lobe (Fig. 2A). Although she received PSL for two weeks, her pulmonary lesions did not improve (Fig. 2B). TBLB of the right B10, but only a few atypical cells were present in the specimens. Two months after discontinuation of the PSL, chest HRCT showed slight worsening of the left lower lobe consolidation (Fig. 2C).
Pathological findings of the surgical lung biopsy. (A) Subpleural perilobular airspace organization and fibrosis (hematoxylin and eosin [HE] staining; panoramic view). (B) Many lymphocytic and plasmocytic infiltrates and lymphatic follicles were present in the lesion. (HE staining; magnification, x 400). (C, D) The alveolar structure was preserved and mural organization was present (Elastica van Gieson staining; magnification, x 400). (E) Subpleural fibroelastosis-like pleuroparenchymal fibroelastosis (HE staining; magnification, x 400). (F) Inflammation in the respiratory bronchioles (HE staining; magnification, x 400).
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The specimen showed subpleural perilobular airspace organization and fibrosis, subpleural fibroelastosis, and inflammation in the respiratory bronchioles (Fig. 3).
(A) Mechanic's hand (arrows). (B) Gottron's papules on the dorsum (arrowheads).
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One month after SLB (6 months after the first visit), cracking and hyperkeratosis of the tips and margins of her fingers (mechanic's hand) and erythematous papules on the dorsum of the right proximal interphalangeal joints and distal interphalangeal joints (Gottron's papules) appeared (Fig. 4).
Pulmonary findings of chest high-resolution computed tomography. (A) One month after the surgical lung biopsy, bilateral subpleural consolidation and ground-glass opacity had worsened. (B) One month and (C) six months after starting the treatment, these findings had improved.
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Chest HRCT showed worsening of the bilateral subpleural consolidation and ground-glass opacity (Fig. 5A). Six months after starting the treatment, chest HRCT showed improvement of the consolidation and ground-glass opacity (Fig. 5B and C).
Clinical course of the patient. BAL: bronchoalveolar lavage, CMV: cytomegalovirus, GCV: ganciclovir, ITCZ: itraconazole, IVCY: intravenous cyclophosphamide, KL-6: Krebs von den Lungen-6, MCFG: micafungin, MDA-5: melanoma differentiation-associated gene 5, PSL: prednisolone, SLB: surgical lung biopsy, TBLB: transbronchial lung biopsy, VRCZ: voriconazole.
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After the treatment was initiated, her clinical symptoms improved, and her KL-6 and ferritin levels decreased (Fig. 6).