Retinitis pigmentosa, Coats disease and uveitis.
OBJECTIVE
To study the anamnestic immune response to retinal specific antigens of two patients suffering from a rare triad of retinitis pigmentosa, Coats disease and uveitis.


METHODS
17-year-old girl presented with an acute episode of panuveitis, and her 19-year-old brother suffered from chronic uveitis. On examination, both patients showed retinal vascular changes and subretinal exudations typical of Coats disease, with bone-spicule pigmentary changes as observed in retinitis 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 the sister's lymphocytes were stimulated with interphotoreceptor binding protein, 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 antigens may play some role in specific types of retinitis pigmentosa. Whether these autoimmune reactions are a primary pathological mechanism or are secondary to the extensive destruction of the photoreceptor layer resulting from the retinitis pigmentosa remains debatable.
Solomon
A
A
Department of Ophthalmology, Hadassah University Hospital, Jerusalem, Israel.
Banin
E
E
Anteby
I
I
Benezra
D
D
D000293:Adolescent; D000328:Adult; D015551:Autoimmunity / Q000276:immunology; D003241:Consanguinity; D005136:Eye Proteins; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000276:immunology; D008297:Male; D015864:Panuveitis / Q000150:complications / Q000276:immunology* / Q000473:pathology; D012173:Retinitis / Q000150:complications / Q000276:immunology* / Q000473:pathology; D012174:Retinitis Pigmentosa / Q000150:complications / Q000276:immunology* / Q000473:pathology; D012177:Retinol-Binding Proteins / Q000276:immunology; D000099214:Retinol-Binding Proteins, Interstitial
D005136:Eye Proteins; D012177:Retinol-Binding Proteins; D000099214:Retinol-Binding Proteins, Interstitial