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A. C. Hogan, S. Jungkim, P. Kenna, D. J. Kilmartin; Baseline Disease Severity and Clinical Course With Immunomodulatory Therapy in Patients With Birdshot Chorioretinopathy. Invest. Ophthalmol. Vis. Sci. 2009;50(13):6033.
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To assess baseline disease severity in Birdshot Chorioretinopathy patients when first receiving immunomodulatory therapy.
Retrospective case series analysis of eleven patients with Birdshot Chorioretinopathy attending a tertiary uveitis clinic. Data collected included demographic details, prior and baseline clinical details, functional assessment with fundus fluorescein angiography, optical coherence tomography, Goldmann visual field, electroretinogram and visual evoked responses, immunomodulatory treatment and clinical course.
Twenty two eyes of eleven patients, seven female and four male, mean age forty two years with a mean follow up of sixteen months (range 7-80) were assessed. At initial referral, Snellen vision was ≥ 6/12 in 91% and 6/6 in 45%; twenty eyes had active intraocular inflammation with choroiditis/vitritis (20), disc swelling (10), cystoid macular oedema (8). At baseline severe optic nerve dysfunction was evident in all eyes with reduced colour vision on ishihara plate testing (14), blind spot enlargement (20) and paracentral scotomas (14) on Goldmann visual field, reduced amplitudes of pattern visual evoked responses (9) and advanced optic atrophy (2). Mean duration to tertiary referral was nine months (range 1-24) and 55% had a previous oral prednisolone pulse. During follow up patients were treated with oral prednisolone (11), mycophenolate mofetil (9, 2 intolerant), tacrolimus (4). Disease relapses (n=19) occurred in 6 patients after achieving clinical inactivity, 58 % attributable to cystoid macular oedema. At latest follow up 73% of eyes had snellen visual acuity ≥ 6/12 and were clinically inactive but all eyes had persistent optic nerve dysfunction.
Despite good visual acuity at baseline all patients had significant optic nerve disease. Although treated appropriately with combination immunosuppression, there was no demonstrable improvement in optic nerve function due to delayed presentation.
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