PMC5348460_01
Chest X rays of our patient at the onset (a) and after the first (b), second (c), and third (e) deteriorations of ILD, showing the peripheral infiltration in the lower lung field and volume loss. A chest X ray taken during remission, between the second and third deteriorations, is shown (d) for comparison.
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A chest X-ray revealed ground glass opacities (GGO) in both lower lung fields (Fig. 1a). Chest X-ray and CT revealed the worsening of peripheral intralobular reticular opacities, bilateral patchy consolidations, and GGOs, which were dominant along the bronchi in the dorsal lung (Fig. 1b, 2b). A chest X-ray revealed the worsening of the bilateral GGOs (Fig. 1c), and chest CT showed peripheral intralobular reticular opacities and subpleural non-segmental patchy GGOs (Fig. 2c). The chest X-ray and chest CT findings showed that she remained in remission at 8 years after the initial visit (Fig. 1d, 2d). A chest X-ray revealed the worsening of the bilateral GGOs (Fig. 1e), while chest CT showed multiple, random, peribronchial consolidation and GGOs that were dominant in the dorsal lung (Fig. 2e).
Chest CT scans of our patient at the onset (a) and after the first (b), second (c), and third (e) deteriorations of ILD, showing peribronchovascular consolidation in the dorsal lungs and non-septal plate-like opacities, intralobular septal thickening, traction bronchiectasis, and peripheral intralobular reticular opacities. The longitudinal levels were not the same, because typical patterns are shown in this figure. A chest CT scan taken during remission, between the second and third deteriorations, is shown for comparison (d).
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Chest computed tomography (CT) revealed bilateral lower consolidation, non-septal plate-like opacity, intralobular septal thickening, and traction bronchiectasis (Fig. 2a). Chest X-ray and CT revealed the worsening of peripheral intralobular reticular opacities, bilateral patchy consolidations, and GGOs, which were dominant along the bronchi in the dorsal lung (Fig. 1b, 2b). The chest X-ray and chest CT findings showed that she remained in remission at 8 years after the initial visit (Fig. 1d, 2d).
The skin symptoms of this patient, showing (a) periungual erythema (arrows) and Gottron's papules (arrowheads), and (b) scaling erythema on the elbows.
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Nine years after the first visit, she again presented with DOE, a high fever, the worsening of Gottron's papules, periungual erythema, the shawl sign, and scaling erythema (Fig. 3).
The disease course of our patient over time. She was initially diagnosed with DM at year 0, and experienced acute deteriorations of ILD during years 1, 4, and 9. After the first deterioration, she was treated with cyclosporine (125 mg/day) plus PSL (30 mg/day). After the second and third deteriorations, she was treated with methylprednisolone (500 mg/day). After the third deterioration, she was also treated with azathioprine, but was later switched to tacrolimus. At the third deterioration, anti-MDA5 antibodies were detected. At the present time, 12 months after the third deterioration, she remains well and is continuing treatment with low-dose corticosteroids and tacrolimus. CyA: cyclosporine, AZA: azathioprine, Tac: tacrolimus
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Her clinical course is shown in Fig. 4.