Long-term follow-up of patients with birdshot retinochoroidopathy treated with corticosteroid-sparing systemic immunomodulatory therapy

Purpose: To evaluate the outcomes of patients with birdshot retinochoroidopathy (BSRC) treated with corticosteroid-sparing systemic immunomodulatory therapy (IMT). Design: Retrospective, noncomparative, interventional case series. Participants: Thirty-five patients with BSRC evaluated at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary from 1980 through 2003. Methods: Data on age, gender, follow-up time, delay to diagnosis or referral, treatment before and during follow-up, complications of BSRC or treatment, Snellen visual acuities (VAs), and electroretinograms (ERGs) were recorded from patient charts. Main Outcome Measures: Disease progression as determined by Snellen VAs and serial ERGs, ocular complications of BSRC or corticosteroids, and complications of systemic IMT. Results: Twenty-eight patients with a mean follow-up of 81.2 months were included. None of the patients had sufficient control of their inflammation before referral. All patients were treated with corticosteroid-sparing systemic IMT at some point during their follow-up: 92.9% were treated with cyclosporine, 67.9% with mycophenolate mofetil, 17.9% with azathioprine, 10.7% with oral methotrexate, and 7.1% with daclizumab. Ocular complications of BSRC and/or corticosteroids were cataract (53.6%), cystoid macular edema (35.7%), glaucoma (21.4%), epiretinal membrane (10.7%), and retinal detachment (3.6%). Average Snellen VAs at the time of initial visit were 0.64 (right eye) and 0.59 (left eye). Average final Snellen VAs were 0.74 (right eye) and 0.71 (left eye). (Logarithm of the minimum angle of resolution equivalents were -0.23, right eye initial; -0.19, right eye final; -0.38, left eye initial; and -0.31, left eye final.) In the right eye, 78.6% of patients and, in the left eye, 89.3% of patients had either the same or improved VA at the end of the follow-up. The 30-hertz flicker implicit time was prolonged in 58.3% of initial ERGs and in 62.5% of final ERGs. The bright scotopic amplitude was abnormal in 45.5% of initial and final ERGs. Conclusions: Long-term preservation of visual function is attainable with systemic corticosteroid-sparing IMT for patients with BSRC. Prompt treatment with systemic IMT may offer the best hope of maintaining retinal function in what is often thought of as a chronically progressive disease resistant to treatment.