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E. Delair, F. Fajnkuchen, D. Bligny, A. Mahr, L. Guillevin, G. Chaine; Differences in Ocular Manifestations between Localized and Generalized Forms of Wegener Granulomatosis: A 44 Cases Study . Invest. Ophthalmol. Vis. Sci. 2003;44(13):2397.
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Purpose:To describe the clinical features and prognosis of ocular manifestations of Wegener’s granulomatosis, with a comparison between localized and generalized forms of the disease. Methods:Retrospective study of 101 cases of Wegener’s granulomatosis between 1986 and 2000. Patients with ocular manifestations were separated in 2 groups depending on the presence or not of renal (respectively generalized and localized forms of the disease). Ocular and extra-ocular clinical features, treatment used, and visual prognosis were evaluated in these 2 groups. Results:44 cases with ocular features were analyzed. 18 (41 %) presented a localized form of the disease and 26 (59 %) a generalized form. Episcleritis was the most common feature (40,9%), followed by conjunctivitis (22,7 %), scleritis (18,2 %), dry-eye syndrome (18,2 %), orbital inflammation (13,7 %), peripheral ulcerative keratitis (13,7 %), anterior uveitis (11,3 %), and other less common manifestations. A difference between the 2 groups was present only for anterior uveitis associated with the generalized form, and for orbital inflammation associated with the localized form. In 10 patients (22,7 %) with decreased visual acuity, 7 presented the localized form of the disease. Most of these patients with severe ocular involvement were treated with intravenous cyclophosphamide and prednisone. For 5 patients, visual acuity improved after treatment. Among the 4 patients with poor visual acuity after treatment, 2 had the generalized form, and 2 had the localized form of the disease. Conclusions:Differences in ocular features were present, depending on the localized or generalized form of Wegener’s granulomatosis. Orbital inflammation was associated with localized forms, whereas anterior uveitis was associated with generalized forms. Decreased vision appeared more often when the disease was localized. However, the final ocular prognosis after treatment was similar for the generalized and localized forms of Wegener’s granulomatosis.
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