AUTOIMMUNE RETINOPATHY IN THE ABSENCE OF CANCER

PURPOSE: To report the clinical and immunologic features of two patien ts with progressive retinal degeneration and circulating antiretinal a ntibodies without systemic malignancy. METHODS: Two patients were foll owed up for 5 to 7 years. Comprehensive medical and ophthalmic examina tions and visual function testing included manual perimetry and standa rdized electroretinography. Patient sera were tested for anti retinal antibodies by Western blot and immunoperoxidase indirect cytochemistry techniques. RESULTS: Two patients had family history of autoimmune di sease. Each had severe monocular visual loss with photopsia, a ring sc otoma, and abnormal electroretinogram despite a normal appearing ocula r fundus. One had a flat electroretinogram; the other had inner retina dysfunction, with selective b-wave loss and abnormal oscillatory pote ntials. Both patients' sera had antiretinal antibodies that specifical ly labeled the inner plexiform layer of donor retina by indirect immun operoxidase testing. Neither had any sign of cancer. CONCLUSIONS: In t wo patients without systemic malignancy, the symptoms, perimetric find ings, and normal fundus appearance resembled cancer-associated retinop athy. Electroretinography and antibody findings indicating dysfunction of the inner retina are distinct from those of cancer-associated reti nopathy. These two cases raise the possibility of an autoimmune mechan ism for retinal degeneration that is not cancer associated. Further st udy is necessary to determine the role of antiretinal antibodies in th ese patients.