Birdshot Retinopathy

Excerpt

Birdshot chorioretinopathy (BCR) is a form of chronic, bilateral, posterior uveitis with a distinguishing clinical phenotype, and a strong association with HLA-A29. This disease predominantly affects individuals of middle-age and more often affects females than males. Early in the course of this disease, patients may present with mild symptoms delaying treatment that can often result in permanent vision changes. This disease is permanent, chronic, and progressive resulting in irreversible ocular damage.

Historically, the first recognition of BCR as a distinct entity was the description by Franceschetti and Babel in 1949 of “candle wax spot chorioretinopathy” in which they reported a 65-year-old woman with discrete depigmented lesionsThe first use of the term “birdshot retinochoroidopathy” was in 1980 when Ryan and Maumenee discussed 13 patients with a particular syndrome characterized by a white, painless eye with minimal anterior segment inflammation, but with vitritis, retinal vascular leakage, and cream-colored spots at the level of the retinal pigment epithelium (RPE) or deeper retinal layers. The condition was further explored and defined by Gass and others. Since the recognition of BCR, physicians have improved treatment options and outcomes. A major breakthrough was recognizing the immunogenicity of the disease and pathogenesis.