Ocular disease in patients with ANCA-positive vasculitis

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis-the term recently applied to Wegener's granulomatosis-is a rare multi-system inflammation characterized by necrotizing granulomas and vasculitis. We investigated the ocular manifestations of this disease in a group of patients drawn from five inflammatory eye disease clinics across the United States. Of 8,562 persons with ocular inflammation, 59 individuals were diagnosed with ANCA-positive vasculitis; 35 males and 21 females, aged 16 to 96 years, were included in this study. Ocular diagnoses were scleritis (75.0%), uveitis (17.9%), and other ocular inflammatory conditions (33.9%) including peripheral ulcerative keratitis and orbital pseudotumor. Mean duration of ocular disease was 4.6 years. Oral corticosteroids and other systemic immunosuppressive agents were used by 85.7% and 78.5% of patients, respectively. Over time, patients with ANCA-positive vasculitis experienced 2.75-fold higher mortality than other patients with inflammatory eye disease.

Fig. 1

Kaplan–Meier survival plot demonstrating probability of survival over time for 7,957 SITE cohort patients who were managed on or before December 2005, and who were residents of the United States. Patients with ANCA-positive vasculitis (n = 56) are represented by the dashed line. Patients with all other diagnoses (n = 7,901) are represented by the solid line