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A. S. Watkins, J. H. Kempen, D. Choi, J. T. Rosenbaum, J. E. Thorne, D. A. Jabs, R. B. Nussenblatt, C. S. Foster, E. B. Suhler, J. R. Smith; Ocular Disease in Patients With Wegener’s Granulomatosis. Invest. Ophthalmol. Vis. Sci. 2008;49(13):800.
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Wegeners granulomatosis is a rare multi-system inflammatory disease characterized by necrotizing granulomas and vasculitis. Results from single center studies suggest that the ocular involvement is aggressive, may result in permanent visual loss and often requires treatment with systemic immunosuppression. We investigated clinical aspects of this disease in a cohort of patients drawn from multiple US tertiary-referral inflammatory eye disease clinics.
The Systemic Immunosuppressive Therapy for Eye Diseases (SITE) database houses retrospectively collected clinical data from the inflammatory eye disease clinics of Johns Hopkins University, the Massachusetts Eye and Ear Infirmary, the National Eye Institute, Oregon Health & Science University and the University of Pennsylvania. We analyzed data collected on patients with Wegeners granulomatosis, including demographic information, disease characteristics, medical interventions and visual outcomes.
Of 7547 persons registered in the SITE database on 11/8/07, 13 males and 25 females aged 16 to 75 years (0.5% of total; 0.05% of patients with uveitis; 3% of patients with scleritis; 12% of patients with orbital inflammatory disease) were diagnosed with Wegeners granulomatosis. Average duration of disease was 4.7 years. Primary ocular diagnoses were scleritis (n=27), orbital inflammation (n=7), uveitis (n=3) and unspecified (n=1). Scleritis subtypes included episcleritis (n=2), anterior (n=17), anterior/posterior (n=2) and necrotizing (n=6). For the 38 patients, disease affected one (n=11) or both (n=20) eyes, or was not specified (n=7). Corrected visual acuity was recorded at least once as ≤ 20/50 for 13 patients (34%) and ≤ 20/200 for 4 patients (11%). Commonest identifiable causes of visual loss were cataract, corneal disease, optic neuropathy and active inflammation. Oral corticosteroids were taken by 37 patients (97%), and treatment with systemic immunosuppression was required in 23 cases (61%).
Wegeners granulomatosis may involve various ocular tissues, most commonly sclera. Our results are consistent with findings of other studies; visual disability and blindness appear to be common, and systemic immunosuppressive medications are frequently used for treatment.
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