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Ocular disease in patients with ANCA-positive vasculitis |
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| Authors: | Angela S. Watkins John H. Kempen Dongseok Choi Teresa L. Liesegang S. S. Pujari Craig Newcomb Robert B. Nussenblatt James T. Rosenbaum Jennifer E. Thorne C. Stephen Foster Douglas A. Jabs Grace A. Levy-Clarke Eric B. Suhler Justine R. Smith |
| Affiliation: | 1. Casey Eye Institute, Oregon Health & Science University, Portland, OR, USA 2. Ocular Inflammation Service, Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA, USA 3. Center for Preventive Ophthalmology and Biostatistics, Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA, USA 4. Center for Clinical Epidemiology and Biostatistics, Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PA, USA 5. Department of Public Health & Preventive Medicine, Oregon Health & Science University, Portland, OR, USA 6. Massachusetts Eye Research and Surgery Institution, Cambridge, MA, USA 7. National Eye Institute, Bethesda, MD, USA 8. Department of Internal Medicine, Oregon Health & Science University, Portland, OR, USA 9. Department of Cell and Developmental Biology, Oregon Health & Science University, Portland, OR, USA 10. Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, MD, USA 11. Department of Epidemiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA 12. Department of Ophthalmology, Harvard Medical School, Boston, MA, USA 13. Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD, USA 14. Department of Ophthalmology, Mount Sinai School of Medicine, New York, NY, USA 15. Department of Medicine, Mount Sinai School of Medicine, New York, NY, USA 16. St. Luke’s Cataract and Laser Institute, Tarpon Springs, FL, USA 17. Portland Veterans’ Affairs Medical Center, Portland, OR, USA 18. Oregon Health & Science University, Mail Code: L467AD, Biomedical Research Building 3181 S.W. Sam Jackson Park Rd., Portland, OR, 97239, USA |
| 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. |
| Keywords: | ANCA-positive vasculitis Wegener''s granulomatosis Eye Scleritis |
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