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BIRDSHOT CHORIORETINOPATHY

BIRDSHOT CHORIORETINOPATHY

Alternative titles; symbolsBSCR▼ Clinical FeaturesBirdshot chorioretinopathy (BSCR) is characterized by multiple small, cream-colored lesions, symmetrically scat...

Alternative titles; symbols

  • BSCR

▼ Clinical Features
Birdshot chorioretinopathy (BSCR) is characterized by multiple small, cream-colored lesions, symmetrically scattered mainly around the optic disc and radiating toward the equator. These depigmented spots, the most distinctive sign of the syndrome, appear at the level of the retinal pigment epithelium but, on occasion, suggest an even deeper infiltration. The retinal vasculopathy and inflammatory signs associated with the funduscopic findings lead regularly to visual loss. Szpak et al. (2001) noted that the human MHC class I specificity HLA-A29 (see 142800) had been observed in nearly all patients (95.8%) with BSCR compared with 7% in healthy controls.

▼ Animal Model
In an attempt to develop an animal model of HLA-A29-associated disease, Szpak et al. (2001) produced transgenic mice expressing HLA-A29 molecules. They found that an eye disorder spontaneously arising in these transgenic mice included many of the features of HLA-A29-associated BSCR in humans.

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