birdshot retinochoroidopathy


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A condition that primarily affects white women, age 40 to 60
Aetiology Unknown. HLA-A29 is present in up to 95%, suggesting a strong immunogenetic predisposition; HLA-A29 has been estimated to increased the relative risk of developing birdshot retinochoroidopathy 200-fold. One series of patients had a strong cell-mediated in vitro response to purified S-antigen, which is found in photoreceptors and causes experimental autoimmune uveitis (EAU) when injected in animals. BRC and EAU share several features including the appearance of deep focal lesions, periphlebitis, and intraocular inflammation, suggesting autoimmunity
DiffDx Pars planitis, APMPPE, serpiginous choroiditis, sarcoidosis, diffuse unilateral subacute neuroretinitis, Behçet’s disease, primary ocular-CNS lymphoma, Whipples disease, multifocal choroiditis and panuveitis, Lyme disease
Management Most cases are resistant to treatment; some cases may respond to steroids, but may recur if discontinued; because of the complications associated with long-term use, steroids are recommended only when visually significant CME or vitritis is present. Administration of periocular steroids may be helpful for the treatment of cystoid macular oedema and avoids unwanted systemic side-effects; other therapies include acetazolamide, cyclosporine in combination with other agents may resolve the macular oedema, reduce vitritis, improve vision, and result in fewer recurrences
Prognosis Most patients experience visual loss to 20/200 or worse in at least one eye; depigmentation of the RPE may be responsible for the vitiliginous spots seen clinically

retinochoroidopathy, birdshot 

A bilateral, chronic condition which appears in middle-aged individuals, over 90% of whom are HLA-A29 positive, suggesting a genetic predisposition. The clinical picture is varied: multiple cream-yellow circular or oval spots spread throughout the posterior fundus, vitritis, cystoid macular oedema and eventually optic atrophy. Choroidal neovascularization (CNV) is a common complication. Symptoms are reduction of visual acuity, floaters, colour vision defects and nyctalopia. Treatment includes corticosteroids and/or immunosuppressants.
References in periodicals archive ?
Crawford, "Histopathology of birdshot retinochoroidopathy," British Journal of Ophthalmology, vol.
Ryan and Maumenee coined the term "birdshot retinochoroidopathy" to describe the distinctive lesions seen in the fundus, characterized by multiple, small, white spots that had the appearance of the scatter from a shotgun (Figure 1) [2].
Foster, "Long-term follow-up of patients with birdshot retinochoroidopathy treated with corticosteroid-sparing systemic immunomodulatory therapy," Ophthalmology, vol.
Kempen, "Birdshot retinochoroidopathy: ocular complications and visual impairment," American Journal of Ophthalmology, vol.
Foster, "Interleukin 21, interleukin 23, and transforming growth factor [beta]1 in HLA-A29-associated birdshot retinochoroidopathy," American Journal of Ophthalmology, vol.
Branley, "Use of intravitreal triamcinolone in the management of birdshot retinochoroidopathy associated with cystoid macular oedema: a case study over a three-year period," Clinical and Experimental Ophthalmology, vol.
Clearly defined uveitis syndromes restricted to the eye19 (e.g., sympathetic ophthalmia, pars planitis, birdshot retinochoroidopathy) with no systemic involvement were included in ocular syndromes.
Among patients who had uveitis with an ocular syndrome, the most frequent cases were those of birdshot retinochoroidopathy (50%) and pars planitis (30%).
Among the Classified group, posterior uveitis was the most common finding followed by panuveitis in which birdshot retinochoroidopathy, tuberculosis and sarcoidosis were mostly prevalent.
These were birdshot retinochoroidopathy followed by pars planitis.
Crawford, "Histopathology of birdshot retinochoroidopathy" British Journal of Ophthalmology, vol.