PMC8320549_01
Chest CT images in Case 1. (A) At the admission. Ground glass opacity and reticular shadows on the peripheral and basilar regions of both lungs were seen. (B) Immediately after the start of the first PE session. Invasive shadows on the dorsal side of the both lungs were seen. In addition, a large cavity with wall thickening was seen in the left lung. (C) After the termination of PE sessions. The ground glass opacity on both lungs still remained, but improved.
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C-reactive protein (CRP) 2.48 mg/dL; and ferritin 208 ng/mL. Chest computed tomography (CT) exhibited ground glass opacity and reticular shadows on the peripheral and basilar regions of both lungs (Figure 1A). In addition, she developed pseudomonas aeruginosa pneumonia which might be due to immunosuppression (Figure 1B), and thus immunosuppressive drugs other than prednisolone were discontinued (Figure 2). KL-6 and AaDO2 also decreased, and RP-ILD, as assessed by chest CT, improved (Figure 1C).
Clinical course of the patient with anti-MDA5 antibody-positive DM-associated RP-ILD (Case 1). The titer of anti-MDA5 antibody (black circle), serum ferritin level (white circle), and serum KL-6 level (black triangle) are shown. The patient was treated with methylprednisolone pulse therapy (mPSL), prednisolone (PSL), tacrolimus (TAC), intravenous administration of cyclophosphamide (IVCY), and rituximab (RTX). Plasma exchange (PE) 3 times a week was initiated in the middle of October, and the frequency of PE was tapered gradually.
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In addition, she developed pseudomonas aeruginosa pneumonia which might be due to immunosuppression (Figure 1B), and thus immunosuppressive drugs other than prednisolone were discontinued (Figure 2). The frequency of PE was tapered gradually, as shown in Figure 2.
PMC8320549_02
Chest CT images in Case 2. (A) At the admission. Ground glass opacity and reticular shadows on both lungs were seen. (B) Before starting the first PE session. The abnormal shadows were deteriorated. (C) After the termination of PE sessions. The ground glass opacity and reticular shadows still remained, but improved.
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Chest CT on admission showed the deterioration of ILD (Figure 3A) compared to the findings a week earlier. However, chest CT showed the exacerbation of RP-ILD (Figure 3B). The CT findings improved (Figure 3C), and the frequency of PE was gradually tapered.
Clinical course of the patient with anti-MDA5 antibody-positive DM-associated RP-ILD (Case 2). The titer of anti-MDA5 antibody (black circle), serum ferritin level (white circle), and serum KL-6 level (black triangle) are shown. The patient was treated with methylprednisolone pulse therapy (mPSL), prednisolone (PSL), tacrolimus (TAC), intravenous administration of cyclophosphamide (IVCY), and rituximab (RTX). Plasma exchange (PE) 3 times a week was initiated in early February, and a total of 31 PE sessions were performed.
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PE 3 times a week was initiated in early February 2020 by using 1920 mL of FFP as the replacement fluid (Figure 4). PE was temporarily performed 4 times a week, because RP-ILD worsened just after the start of PE.