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Comment & Response
March 14, 2019

Implications of Birdshot-Like Uveitis on the Pathogenesis of Birdshot Chorioretinopathy

Author Affiliations
  • 1Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles
JAMA Ophthalmol. 2019;137(5):583-584. doi:10.1001/jamaophthalmol.2019.0214

To the Editor The recent article by Acaba-Berrocal et al1 presents a case of birdshot-like chorioretinopathy in a patient who was HLA-A29–negative and received treatment with a checkpoint-blockade agent for malignant melanoma. This case is of considerable interest with implications for the immunopathogenesis of birdshot chorioretinopathy (BCR).

In most, if not all, patients with BCR, HLA-A29 is found; however, HLA-A29 only confers risk, in that most white individuals who are HLA-A29–positive do not develop BCR. Increased risk is also conferred by killer-cell immunoglobulin-like receptor genes and the HLA-B44 subtype that interacts with killer-cell immunoglobulin-like receptors,2 suggesting the possibility of either an infectious or neoplastic trigger. We believe the inciting event may be the presence of skin cancers or precancerous lesions, which are highly prevalent in middle-aged white individuals, the population in which BCR is almost exclusively found. There is also a rare association of BCR with ocular melanoma, further supporting a role for neoplasia inciting the disease.3 In the case presented by Acaba-Berrocal et al,1 we propose that immune activation through release of inhibition of the programmed cell death protein 1 checkpoint pathway in a patient treated for cutaneous melanoma mimics the altered immune reactivity to neoplastic changes in the skin (whether manifest or microscopic) in a subset of individuals who are HLA-A29–positive and develop BCR.

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