Ocular Manifestations at Diagnosis of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Clinical Implications from a Retrospective Cohort.

OBJECTIVE: Ocular involvement is an important source of morbidity in anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis (AAV) and may occasionally be the initial manifestation. This study evaluated the frequency, clinical characteristics, and systemic associations of ocular involvement in patients with AAV. METHODS: In this retrospective cohort study, 105 patients with AAV followed at the Division of Rheumatology, University of Health Sciences Gülhane Training and Research Hospital between November 2016 and July 2024 were analyzed. Demographic, clinical, ANCA profile, vasculitis subtype, and organ involvement data were obtained from medical records. Patients were grouped according to ocular involvement at diagnosis, and clinical and laboratory characteristics were compared. RESULTS: Ocular involvement was identified in 22 patients (21.0%). Twenty-one patients (95.5%) had granulomatosis with polyangiitis, and 18 (81.8%) were proteinase 3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA) positive. Episcleritis (n = 12, 54.5%) and scleritis (n = 4, 18.2%) were the most common manifestations. Less frequent findings included anterior uveitis (n = 4, 18.2%), ischemic optic neuropathy (n = 1, 4.5%), and peripheral ulcerative keratitis associated with scleritis (n = 1, 4.5%). No significant differences were observed in demographic characteristics, disease activity, ANCA subtype distribution, or systemic organ involvement between groups. CONCLUSION: Ocular involvement is clinically relevant in AAV at diagnosis and may represent the initial presentation of systemic vasculitis. Episcleritis and scleritis were the most common findings, whereas vision-threatening complications were uncommon. Early recognition and ophthalmology-rheumatology collaboration may facilitate timely diagnosis and management.