PMC9726841_01
Physical and imaging findings at the time of her admission. (a) Photographs of her hands and fingers. They show Gottron's sign and reverse Gottron's sign, periungual erythema, and pre-ulcerative lesion of fingertips. (b) Computed tomography images of the chest. Reticular shadows in bilateral lower lobes, just below the pleura, and around bronchovascular bundles, a decrease in the volume of the bilateral lower lungs, and multiple consolidations were shown. (c) The non-contrast MRI of the lower extremes. Her left semitendinosus and biceps femoris showed high intensity in short T1 inversion recovery (STIR) (yellow arrowhead). (d) Maximum intensity projection (MIP) imaging using slice-stacking contrast-enhanced magnetic resonance imaging (MRI) of her right hand. Her PIP, MCP, and wrist joints are enhanced and imply the presence of synovitis.
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The photographs of her hands and fingers are shown in Figure 1(a). Her chest CT images are shown in Figure 1(b), which indicated the non-specific interstitial pneumonia (NSIP) with organizing pneumonia variant. The non-contrast magnetic resonance imaging (MRI) of the lower extremes is shown in Figure 1(c). Muscle-skeletal echography showed high-power Doppler and maximum intensity projection (MIP) imaging using slice-stacking contrast-enhanced MRI of her right hand enhanced and imply the presence of synovitis on her proximal interphalangeal, metacarpophalangeal, and wrist joints (Figure 1(d)).
Therapeutic intervention and clinical course of treatment. (a) The numbers displayed on the horizontal axis represent days. Triple therapy was started on day 1. The dose of PSL was reduced by 5 mg every 2 weeks. The dose of PSL reached 40 mg daily on day 26. Her skin lesions became worsened and the dose of PSL elevated up to 50 mg daily on day 28. MMF was added at day 33 and continued for 2 weeks. Peficitinib was started on day 49 and continues to the present. IVCY was administered on day 1, 31, 59, and 87 (PSL: prednisolone; TAC: tacrolimus; IVCY: intravenous cyclophosphamide; MMF: mycophenolate mofetil). (b) Transition of skin lesions and chest CT images from admission to 1 year after treatment. They improved over time.
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Her clinical course of treatment is shown in Figure 2. The titer of anti-MDA5 Ab and KL-6 continued to drop even though the PSL had been tapering and IVCY was completed in four times as indicated by the green arrow in Figure 2.