Ocular involvement in antineutrophil cytoplasmic antibody-associated vasculitis

Ocular involvement is a common and potentially disabling manifestation of antineutrophil cytoplasmic antibody-associated systemic vasculitis (AAV). Available data on the frequency and pattern of ocular involvement are focused mainly on granulomatosis with polyangiitis (GPA), whereas they remain insufficiently studied in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA).

Objective: to analyze the frequency and patterns of ocular involvement in GPA compared with other AAV.

Material and methods. Data from 174 patients with AAV were analyzed, including 113 with GPA, 35 with MPA, and 26 with EGPA, who were followed up at the V.A. Nasonova Research Institute of Rheumatology from 2023 to 2025. Demographic and clinical parameters were analyzed.

Results and discussion. Ocular involvement was identified in 55 (31.6%) of 174 patients, most often in GPA (n=50, 44.2%), less often in MPA (n=4, 11.4%) and EGPA (n=1, 3.8%). The most common manifestations were episcleritis/scleritis (n=36, 65.5%), peripheral corneal ulceration (n=11, 20%), uveitis (n=16, 29%), and orbital pseudotumor (n=13, 23.6%). In 31 (56.4%) patients, one type of ocular involvement was present; in 24 (43.6%), two or more. Ocular involvement correlated positively with anti-proteinase 3 (PR3)-ANCA levels and negatively with anti-myeloperoxidase (MPO)-ANCA concentrations. The most common ocular complication was cataract (n=23, 42%). Irreversible vision loss was observed only in patients with GPA (n=5, 4.4%).

Conclusion. Ocular involvement was detected in one third of patients with AAV, especially in GPA with PR3-ANCA-positive status. The frequency of irreversible vision loss remains high.

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