Immune response to retinal antigens in patients with gyrate atrophy and other hereditary retinal dystrophies

Purpose: Gyrate atrophy (GA) is a rare hereditary disease that causes retin al destruction. Retinal damage in GA and other heredodegenerative diseases such as retinitis pigmentosa (RP) releases sequestered antigens and may tri gger immune response to these molecules. Here, we studied the immune respon se to retinal antigens in patients with GA and RP and compared it with that of patients with inactive posterior uveitis and normal volunteers. Patient s and methods: Peripheral blood was collected from 24 patients with RP, 10 patients with GA, 10 patients with inactive posterior uveitis, and 16 norma l volunteers. Cell-mediated immune responses to human S-antigen (HS-Ag), bo vine S-antigen (BS-Ag), and interphotoreceptor retinoid-binding protein (IR BP) were investigated by lymphocyte proliferation assay. In addition, serum levels of soluble intercellular adhesion molecule-r (sICAM-I) and soluble vascular cell adhesion molecule-I (sVCAM-I) were studied by ELISA. Immunolo gic data were correlated with clinical and electrophysiological findings. R esults: Patients with GA or RP responded to HS-Ag and BS-Ag more vigorously than patients with uveitis or healthy controls, as shown by higher mean st imulation indices and larger proportions of responders. Unlike S-Ag, IRBP s timulated low lymphocyte responses in only a small proportion of RP patient s. The mean sVCAM-I levels were significantly higher in the sera from patie nts with GA than in that from normal controls. Conclusion: An elevated cell ular immune response to S-Ag is common in patients with GA and RP. This ele vated cellular immune response to S-Ag may exacerbate retinal destruction i n patients with GA and RP.