Photoreceptor degeneration. Possible autoimmune disorder

Abstract

A 61-year-old woman with progressive blindness, initially responsive to steroid therapy, was found to have an undifferentiated neoplasm in the cervix uteri. Visual fields demonstrated ring scotomas and the electroretinographic pattern eventually became flat. The interval from onset of visual symptoms to blindness was 19 months. Eye pathologic findings included loss of retinal photoreceptors, a macular hole, and normal optic nerves. Antibodies were demonstrated in the patient's serum to normal fresh human retinal photoreceptors. An autoimmune mechanism may have been responsible for the retinal degeneration in this patient, and such a hypothesis raises questions about (1) autoimmune mechanisms in patients with retinitis pigmentosa and other retinal degenerative diseases and (2) autoimmune mechanisms in other cases of remote effects of carcinoma.