case_images: PMC10474959_01
case_images: Clinical course of MPO-ANCA and C-reactive protein. During her clinical course, AAV and scleritis relapsed twice, at 65 months and 53 months before the current admission. At 65 and 53 months before the current admission, the MPO-ANCA titer was in the positive range and obviously increased, and C-reactive protein levels were also increased, although the C-reactive protein level was low, at less than 1.0 mg/dL (green circle). At the time of admission, C-reactive protein levels did not obviously increase (green circles). In addition, although the MPO-ANCA titer at the time of admission was positive, the titer was not obviously increased compared to the levels over the previous several months.
case_images: CRIRH2023-4246075.001.jpg
case_images: PMC10474959_01_CRIRH2023-4246075.001.jpg
case_images: fig1
case_images: A 74-year-old woman developed bilateral hearing loss, scleritis, positivity for myeloperoxidase (MPO)-ANCA (the MPO-ANCA titer was >300 U/mL; the clinical course of MPO-ANCA, and C-reactive protein is presented in Figure 1), and hematuria (occult blood 2+ and red blood cells >30-49 per high-power field in sedimentation) seven years ago. Her hearing loss, scleritis, and urinary abnormalities gradually improved (Figure 2(a)) and the MPO-ANCA titer also decreased (Figure 1). AAV, were not detected. Although her MPO-ANCA titer at admission was in the positive range, it did not obviously increase compared to the levels over the several previous months (Figures 1 and 3).
case_images: Right eye findings during the clinical course of the case. The clinical course of the findings in the right eye. After the initial immunosuppressive therapy at the time of the first AAV development, the scleritis improved. (a) The right eye findings two months before the current hospital admission. Corneal ulcers (arrows). (b) Right eye findings at the time of current hospital admission. Hyperemia in the sclera indicated scleritis (circle), corneal ulcer progression, and perforation (arrow heads) (c).
case_images: CRIRH2023-4246075.002.jpg
case_images: PMC10474959_01_CRIRH2023-4246075.002.jpg
case_images: fig2
case_images: Her hearing loss, scleritis, and urinary abnormalities gradually improved (Figure 2(a)) and the MPO-ANCA titer also decreased (Figure 1). She was diagnosed with scleritis with a corneal ulcer (Figure 2(b)), and because the current ocular lesion was considered to be due to AAV relapse and infectious conditions were not detected, the PSL dose was increased to 30 mg/day. Two months later, the ocular lesion worsened, and she was diagnosed with aggravation of the right scleritis with corneal perforation (Figure 2(c)).
case_images: Clinical course of AAV treatment and MPO-ANCA and C-reactive protein titer changes. After the surgery, methylprednisolone 500 mg pulse therapy was administered for three days, and 30 mg/day of oral PSL was started. Rituximab (500 mg) was then administered twice. The MPO-ANCA titer decreased to 10.0 U/mL. The patient was discharged after the PSL dose was reduced to 20 mg/day.
case_images: CRIRH2023-4246075.003.jpg
case_images: PMC10474959_01_CRIRH2023-4246075.003.jpg
case_images: fig3
case_images: AAV, were not detected. Although her MPO-ANCA titer at admission was in the positive range, it did not obviously increase compared to the levels over the several previous months (Figures 1 and 3).