Birdshot Chorioretinopathy is the disease with the strongest association to a HLA class I antigen, with more than 95% of patients carrying the HLA-A29 antigen. The relative risk of HLA-A29 carriers developing Birdshot Chorioretinopathy has been estimated to be between 50 and 250. HLA-A*29:02, which is the most frequent A29 allele in the Caucasian population is also the allele most frequently associated with Birdshot Chorioretinopathy in Caucasians. The disease has however been observed in HLA*29:01 Caucasian patients. Birdshot Chorioretinopathy is extremely rare in Asia where the most frequent HLA-A29 allele is A*29:01.
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