Birdshot retinochoroidopathy
- National Eye Institute, National Institutes of Health, Bethesda, MD, USA
- Scott M Whitcup, MD, National Eye Institute, National Institutes of Health, 10 Center Drive, Bldg 10 Rm 10N202, Bethesda, MD 20892–1858, USA.
Birdshot retinochoroidopathy is a rare, chronic, bilateral, posterior uveitis with a distinctive clinical presentation and a strong genetic association. Middle aged white people of northern European extraction are most commonly afflicted.1-4 The pathogenesis is unknown, but HLA-A29 positivity appears to confer predisposition,5-9 and retinal autoimmunity seems to play a role.8
This review provides an updated summary of the current state of knowledge about birdshot retinochoroidopathy. It includes results of visual field and electrophysiological testing from markedly more patients than previous reports of these data. Furthermore, it provides prognostic information regarding visual acuity based on nearly twice the number of patients than the one previous report of this aspect of the disease. Such information is of special interest to patients. The paper is based on a chart review of the 59 birdshot patients evaluated at the National Eye Institute (NEI) and from a comprehensive review of the literature.
Historical background
Perhaps the earliest report of birdshot retinochoroidopathy was in 1949 by Franceschetti and Babel, who named it descriptively “la chorio-rétinite en ‘täches de bougie’”—candle wax spot chorioretinopathy.10 Subsequently, other descriptive terms have been used—salmon patch choroidopathy,11“choriorétinopathie en grains de riz” (that is, rice grain chorioretinopathy),12 vitiliginous chorioretinitis,1 and birdshot retinochoroidopathy.4 The term “vitiliginous chorioretinitis” was chosen both because the hypopigmented fundus lesions resemble vitiligo and because some individuals in one cohort of birdshot patients had cutaneous vitiligo. However, this systemic association has been noted by only one researcher.1 The term “birdshot retinochoroidopathy” is most commonly used. It was coined by Ryan and Maumenee in 1980 to describe the fundus lesions, which typically consist of “multiple, small, white spots that frequently have the pattern seen with birdshot in the scatter from a shotgun.”4 The disease became accepted as a distinct entity in the …
