Birdshot Retinochoroidopathy

Birdshot Retinochoroidopathy (BSRC) is a rare ocular disorder that is characterized by bilateral chronic intraocular inflammation and multiple, discrete, cream-colored foci of depigmentation scattered diffusely throughout the fundus. These hypopigmented lesions often have a vascular orientation. The disease has exacerbations and remissions and can lead to markedly reduced visual acuity.

The etiology and pathogenesis of BSRC are unknown. It is strongly associated with HLA-A29 suggesting a greater predisposition. Autoimmunity to retinal soluble protein may play a role in the perpetuation of the intraocular inflammation.

Other terms for this have been vitiligenous chorioretinitis, salmon patch choroidopathy, and candle wax spots.

The differential diagnosis includes pars planitis, intraocular primary large B cell lymphoma, syphilitic chorioretinitis, multiple evanescent white dot syndrome, punctate inner choroidopathy, and sarcoidosis.

Treatment with oral steroids supplemented with sub-Tenon injections of depot steroids for various time periods have been used. The determination of visual acuity is used to gauge the treatment efficacy and to determine the appropriate dose or regime of steroids for each patient and for the course of the disease. Initial use of 40 to 60 mg and tapering to 5 mg or less daily for several months may be necessary. Low dose cyclosporine may be useful in cases refractory to steroid therapy.

BSRC is an ocular inflammatory disease with a genetic predisposition.