Birdshot chorioretinopathy

Birdshot chorioretinopathy (also known as birdshot retinochoroidopathy or vitiliginous chorioretinitis) is a rare, chronic, bilateral inflammatory eye disease that primarily affects the choroid and retina. It is classified as a posterior uveitis and is characterized by the appearance of distinctive cream-colored or depigmented ovoid lesions scattered across the fundus (back of the eye) in a pattern resembling birdshot scatter from a shotgun, which gives the disease its name. The condition predominantly affects middle-aged Caucasian individuals, typically presenting in the fourth to sixth decade of life, and has a strong genetic association with the HLA-A29 antigen, which is found in over 90% of affected patients. Key symptoms include progressive blurred vision, floaters, nyctalopia (difficulty seeing in dim light), dyschromatopsia (impaired color vision), photopsia (flashes of light), and reduced contrast sensitivity. Patients often experience vitritis (inflammation of the vitreous humor), retinal vasculitis, cystoid macular edema, and optic disc edema. Over time, the disease can lead to significant visual impairment if left untreated, with complications including macular edema, optic atrophy, epiretinal membrane formation, and choroidal neovascularization. Treatment aims to control intraocular inflammation and preserve visual function. Corticosteroids may be used initially, but long-term management typically requires steroid-sparing immunosuppressive agents such as mycophenolate mofetil, cyclosporine, methotrexate, or biologic therapies including adalimumab and infliximab. Local treatments such as intravitreal corticosteroid implants may also be employed. Early and sustained treatment is important to prevent irreversible retinal damage and vision loss. The disease follows a chronic, progressive course, and regular monitoring with electroretinography (ERG), optical coherence tomography (OCT), and fluorescein angiography is essential for managing the condition effectively.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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