A Case of an Elderly Woman Who Developed Corneal Perforation in the Clinical Course of Myeloperoxidase Positive Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Abstract

Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a systemic vasculitis characterized by ANCA positivity and categorized into three main types: microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatous with polyangiitis. Although AAV leads to systemic organ injury, such as of the lungs, kidneys, nerves, and skin, patients with AAV sometimes develop ocular lesions. Here, we report the case of an elderly woman who had been treated for AAV for seven years. She developed scleritis and relapsed twice, with elevation of serum disease markers such as ANCA titer and C-reactive protein. After the decline of these markers due to treatment with additional medication, her scleritis relapsed again and caused a corneal ulcer, which resulted in perforation without obvious marker elevation. She did not present with any symptoms of organ injury, except for ocular lesions. She was treated with surgery, followed by methylprednisolone and rituximab therapy. Subsequently, her ocular lesions and symptoms improved, and she did not relapse. AAV can cause various ocular manifestations. Although C-reactive protein and ANCA titers are useful markers of disease activity and the relapse of AAV complications, including ocular lesions, these markers do not always increase at the time of worsening ocular lesions. Therefore, it is important for clinicians treating patients with AAV to pay careful attention to serum data and physical findings, including the eyes.

Figure 1

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.

Figure 2

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).

Figure 3

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.