HLA-A29-Associated Retinal Vasculitis Without Choroidal Lesions

Abstract

Purpose : Birdshot retinochoroidopathy (BSRC) is characterized by multifocal choroidal lesions, retinal vasculitis, and macular edema. However, some patients may present with HLA-A29-associated retinal vasculitis without choroidal lesions. We aim to test the hypothesis that these patients may share clinical features with birdshot retinochoroiditis, including the need for systemic immunosuppressive therapy.

Methods : A retrospective medical chart review of patients who were HLA-A29 positive and had retinal vasculitis, yet no birdshot lesions was performed. Demographic, clinical, and therapeutic data were entered into a new database for analysis.

Results : Five patients who had HLA-A29-associated retinal vasculitis without choroidal lesions were identified. The median age at presentation was 52 years (range 14 - 71); 60% were female. At presentation, four patients had a visual acuity of 20/50 or better in both eyes. All eyes had mild vitritis, while 3 eyes (30%) had cystoid macular edema. One patient presented with bilateral optic nerve swelling. All patients required treatment with systemic steroids and immunosuppressive therapy.

Conclusions : HLA-A29-associated retinal vasculitis without choroidal lesions appears to share many clinical features with birdshot chorioretinitis, including the need for systemic immunosuppressive therapy. Whether this entity represents an early form of birdshot retinochoroiditis or a more localized variant of the disease is a topic for further studies.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.