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A. Wissen, M. J. Saeger, J. Roider, B. Noelle; Severe Cases of Occlusive Retinal Vasculitis. Invest. Ophthalmol. Vis. Sci. 2010;51(13):5850.
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Retinal vascular occlusion is a rare finding in patients with uveitis. Both idiopathic occlusive vasculitis and an association with systemic diseases such as Wegener’s granulomatosis, Behcet’s syndrome, systemic lupus erythematosus and sarcoidosis are known. Initiation of an immunosuppressive treatment as well as early laser treatment of the retina may be useful.
We present a retrospective case series of 13 eyes of 9 patients presented with severe occlusive retinal vasculitis to our hospital between 2006 and 2009.
The average age was 44 years (range 21 - 68). 7 patients were male, 2 female. In 4 patients both eyes were affected. BCVA was between <0,13 and 0,0 logMAR depending on the localization of retinal ischemia. Ophthalmoscopy and fluorescein angiography showed severe retinal periphlebitis, haemorrhages and areas of retinal non-perfusion. There was no prior history of autoimmune diseases. All patients received laser treatment of ischemic retina. 7/9 patients were treated with systemic and topical corticosteroids. Two patients were diagnosed with sarcoidosis, one patient with Behcet’s syndrome and one with systemic lupus erythematosus. An immunosuppressive therapy including methotrexate, ciclosporine or interferon was started in 4 of them. 5 patients showed an idiopathic occlusive vasculitis without any systemic association. An HLA-typing was performed in 4 patients without relevant findings. Immune complexes and complement factors were elevated: CIC-IgG (4/9), CIC-IgM (4/9), CIC-C4 (3/9), CIC-C1q (1/9), factor 3 (1/9). Two patients found to be ANA positive, one patient ANCA-IF positive. 6/9 patients experienced a recurrent occlusive event while reducing systemic glucocorticosteroids. 5 patients developed vitreous haemorrhage. Vitrectomy was performed in two cases. Two patients presented secondary glaucoma. Follow-up (4-20 months) revealed quiet condition with neither vitreous cells or neovascularizations nor progressing ischemia in 8/9 patients.
Vaso-occlusive retinopathy may present idiopathically (5/9) or in association with autoimmune diseases (4/9). Eye inflammation can be the first sign of a systemic autoimmune disease. An apparent correlation to HLA antigens is not seen, circulating immune complexes are frequently elevated. Early laser treatment of the retina, systemic corticosteroids, and systemic immunosuppressive drugs enable a successful course.
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