Retinitis pigmentosa, Coats disease and uveitis

Purpose. To study the anamnestic immune response to retinal specific antige ns of two patients suffering from a rare triad of retinitis pigmentosa, Coa ts disease and uveitis. Patients. 17-year-old girl presented with an acute episode of panuveitis, a nd her 19-year-old brother suffered from chronic uveitis. On examination, b oth patients showed retinal vascular changes and subretinal exudations typi cal of Coats disease, with bone-spicule pigmentary changes as observed in r etinitis pigmentosa. Results. All routine examinations were unrevealing. However, the peripheral lymphocytes from these two siblings gave a specific anamnestic response to retinal antigens in vitro. A stimulation index of 4.6 was obtained when th e sister's lymphocytes were stimulated with interphotoreceptor binding prot ein. IRBP-during the acute stage of the uveitis. The brother's lymphocytes showed a stimulation index of 2.7 towards S-Ag during the chronic phase of his uveitic condition. Conclusions. These results indicate that autoimmunity towards retinal antig ens may play some role in specific types of retinitis pigmentosa. Whether t hese autoimmune reactions are a primary pathological mechanism or are secon dary to the extensive destruction of the photoreceptor layer resulting from the retinitis pigmentosa remains debatable.