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Homaira Ayesha Hossain, C Stephen Foster; Autoimmune retinopathy in patients with birdshot retinochoroidopathy (BSRC) who are in remission. Invest. Ophthalmol. Vis. Sci. 2014;55(13):2510.
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To investigate the continued deterioration of vision in patients with BSRC who are in remission from active uveitis.
We identified four patients who demonstrated continued deterioration in visual function despite long-term remission from active BSRC as evinced by absence of inflammation on clinical ophthalmoscopic examination and fluorescein angiogram. This visual decline resembled that of autoimmune retinopathy, with painless loss of vision and decline in visual acuity, abnormal electroretinography, and defects on visual field testing. Sera were tested for anti-retinal and anti-optic nerve antibodies by Western blot analysis.
The four patients included two males and two females, with an average age of 60.3 years (ranging from 54 to 68 years). All patients were HLA-A29 positive. Three patients were treated with a minimum of two years of immunomodulatory therapy (IMT). One patient was treated with IMT for five months, after which it was discontinued due to medication side effects. She subsequently underwent bilateral fluocinolone acetonide intravitreal implants. At the time of serum testing, all four patients demonstrated absence of inflammation on clinical ophthalmoscopic examination and fluorescein angiogram. Two patients demonstrated visual decline as evinced by both visual field testing (SITA-SWAP) and electroretinography (decreased amplitude and/or increased implicit time on 30 Hz flicker). One patient demonstrated visual decline in electroretinography testing only, and one patient demonstrated visual decline in visual field testing only. The serum of all four patients demonstrated positivity for anti-retinal antibodies. The serum of three out of four patients demonstrated positivity for anti-optic nerve antibodies.
The presence of anti-retinal and anti-optic nerve antibodies in patients who have achieved long-term remission of BSRC after prolonged treatment suggests that continued deterioration has occurred because of autoimmune retinopathy. Patients with BSRC who present with visual decline but show no active inflammation on clinical ophthalmoscopic examination and fluorescein angiogram may benefit from autoantibody screening. For these patients, further treatment with IMT may be indicated due to the presence of autoantibodies implicated in the continued deterioration of visual function.
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