Retinal vasculitis associated with autoantibodies to Sjögren's syndrome A antigen

Severe retinal vasculitis caused by a systemic lupus-like illness developed in two patients with distinctive clinical and immunologic characteristics. Both patients were young women with mild systemic disease and autoantibodies directed against a protein-ribonucleic acid complex termed the Sjögren's syndrome A antigen (SSA). One patient had no antibodies to nuclear antigens on conventional testing, and the other had a low-titer antinuclear antibody test. At the time of onset of retinal arteriolitis, neither patient had an exacerbation of multisystem disease or serologic activity. Despite oral and parenteral corticosteroids, cytotoxic agents, and panretinal photocoagulation, both patients suffered progressive irreversible retinal ischemia, optic disk and retinal neovascularization, vitreous hemorrhage traction retinal detachment, and anterior segment neovascularization.