Purpose: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is an intractable disease, is a systemic necrotizing vasculitis characterized by high ANCA positivity. In cases of ANCA, the primary ocular complication is scleritis; however, other complications such as orbital and uveal lesions, which are intractable, are often observed. In this study, we investigated ANCA-positive patients with scleral lesions.
Subjects and methods: We examined a total of 12 eyes of 10 ANCA-positive patients with scleral lesions (3 men and 7 women; mean age: 76.8±9.4 years; range: 58-88 years) who visited the Department of Ophthalmology, Kyoto Prefectural University of Medicine Hospital, between 2009 and 2019. In all subjects, corrected visual activity (VA), treatments administered, and ocular complications were investigated retrospectively.
Results: Of the 12 treated eyes, 11 (92%) had scleritis, 1 (8%) had scleral thinning without inflammation, 2 (17%) had orbital lesions, and 4 (33%) had uveitis. In 2 (20%) patients with scleritis, the disease was binocular. Best corrected VA at initial presentation and final follow-up visit was 0.13±0.21 and 0.55±0.96, respectively (p=0.168). For treatment, all 10 patients were prescribed topical steroid eye drops, and 9 (90%) were prescribed oral steroids or immunosuppressants. Of the 10 eyes that could follow the treatment course, decreased corrected VA was observed in three eyes, and uveitis occurred in these patients.
Conclusion: Patients with ANCA-associated vasculitis are at a high risk of scleral complications; therefore, strict attention must be paid to decreased corrected VA, particularly in patients with uveitis.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 126: 697-702,2022.