| | Age | Sex | ANCA type | ANCA titer | CRP (mg/dL) | Ocular manifestation | Organ involvement | Treatment |
| [16] | 52 | M | P | Positive | 16.7 | Scleritis | Neuropathy | PSL, mPSL, CY, MTX | [16] | 59 | F | P | 219 EU | >10 | Scleritis | Kidney | PSL | [16] | 42 | M | P | >640 EU | 3.1 | Scleritis | Lung | PSL, mPSL, CY | [21] | 73 | F | MPO | 235 U/mL | 10.4 | Bilateral orbital apex syndrome | ENT | PSL, mPSL, MTX | [22] | 69 | F | MPO | 124 RU/mL | 0.4 | Scleritis | ENT | PSL, mPSL, RTX, AZA | Current case | 74 | F | MPO | 22.0 U/mL | 0.01 | Scleritis, cornea ulcer, perforation | — | PSL, mPSL, MZR, RTX | [17] | 54 | M | C | Positive | Elevated | Necrotizing scleritis, uveitis | ENT | PSL, mPSL, MTX | [17] | 56 | M | C | Positive | 16.8 | Necrotizing scleritis, ulcerative keratitis | — | PSL, mPSL, CY | [17] | 50 | F | C | Positive | 13.7 | Necrotizing scleritis, ulcerative keratitis, cornea ulcer | Lung | PSL, mPSL, CY, MTX | [17] | 30 | M | C | Positive | 43.0 | Necrotizing scleritis, ulcerative keratitis | ENT, lung, kidney | PSL, CY, IVCY | [12] | 37 | M | C | Positive | 7.7 | Scleritis, corneal perforation conjunctivitis | ENT, lung | IVCY, CY, mPSL | [23] | 38 | M | C | Positive | 7.2 | Conjunctivitis, episcleritis, anterior uveitis | Kidney | PSL, mPSL, RTX, PE | [24] | 53 | F | PR3 | 175 IU/mL | 1.2 | Episcleritis | Kidney, muscle, joint | PSL, CY, AZA | [19] | 67 | M | PR3 | 23 EU/mL | 0.2 | Scleritis | ENT | PSL, mPSL, IVCY, RTX | [25] | 44 | F | PR3 | 5.9 U/mL | 0.12 | Scleritis | ENT, skin | PSL, mPSL | [26] | 73 | F | PR3 | 725 U/mL | 0.4 | Scleritis | Kidney, lung | PSL, RTX | [18] | 40 | F | PR3 | 195 AU/mL | 6.6 | Ulcerative keratitis | ENT, lung | PSL, CY | [20] | 19 | F | PR3 | Positive | 11.0 | Necrotizing scleritis, ulcerative keratitis | ENT | PSL, AZA, RTX, MTX | [27] | 74 | M | P and C | 28 EU/mL and 23 EU/mL | 0.05 | Scleritis | — | PSL, mPSL |
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AAV: antineutrophil cytoplasmic antibody-associated vasculitis, ANCA: antineutrophil cytoplasmic antibody, AZA: azathioprine, CRP: C-reactive protein, CY: cyclophosphamide, ENT: ear, nose, and throat, ESR: erythrocyte sediment rate, F: female, IVCY: intravenous cyclophosphamide, M: male, MPO: myeloperoxidase, mPSL: methylprednisolone, MTX: methotrexate, PE: plasma exchange, PR3: proteinase 3, PSL: prednisolone, RTX: rituximab.
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