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Ramana S. Moorthy, LIk Thai Lim, Albert T. Vitale, Vinaya Vasudevan, Julie Shelton; Macular Retinal Vasculitis And Choroiditis Associated With Wegener Granulomatosis. Invest. Ophthalmol. Vis. Sci. 2012;53(14):3214. doi: https://doi.org/.
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To report two cases of Wegner granulomatosis (WG) which developed macular retinal vasculitis and choroiditis, not previously documented to be associated with WG, healed with fibrosis and severe vision loss, despite aggressive immune-modulating therapy (IMT) and good control of systemic disease.
An observational case series of the progress of two cases of WG necrotizing retinal vasculitis and choroiditis of the macula.
In the first case (unilateral) of WG, the presenting visual acuity was counting fingers (CF) at one meter left eye. The second case presented bilaterally with visual acuity of 20/200 in the right eye and 20/25 in the left eye at 20/25 (lesion temporal to macula). Both cases of WG associated macular retinal vasculitis and choroiditis were characterized by a central or paracentral yellow-white infiltrative lesion with variable amounts of retinal hemorrhage, choroidal thickening, and mild vitritis. Spectral domain OCT in the second case suggested outer retinal disruption, subretinal fluid, and thickened choroid. Fluorescein and ICG angiography confirmed retinal vascular and choroidal inflammation. Ab interno retinal biopsy in both cases demonstrated mixed inflammatory cells with no infectious etiology. Despite systemic steroids and aggressive IMT (including azathioprine, methotrexate and cyclosporine), the end result in both cases was macular fibrosis with poor visual outcome.
In conclusion, WG can be associated with macular retinal vasculitis and choroiditis leading to fibrosis accompanied by poor visual prognosis.
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