CILIORETINAL ARTERY-OCCLUSION IN SICKLE-CELL TRAIT AND RHEUMATOID-ARTHRITIS

Background: Although once thought to be a benign condition, retinal va scular occlusive disease and proliferative retinopathy can occur with sickle cell trait (hemoglobin AS) when additional systemic diseases or trauma are present. Methods: The authors discuss the ophthalmologic e valuation and clinical course of a 49-year-old woman with sickle cell trait and rheumatoid arthritis who presented with a cilioretinal arter y occlusion. Results: The patient's laboratory evaluation showed both a high rheumatoid factor titer and a mild hypergammaglobulinemia, caus ing increased serum viscosity. The high level of sickle hemoglobin-42. 3% (range in trait, 22%-46%)-increased serum viscosity, and lower cili oretinal artery perfusion pressure relative to the central retinal art ery resulted in cilioretinal artery occlusion. Conclusions: Isolated c ilioretinal artery occlusions carry a good prognosis, and this patient recovered 20/20 visual acuity in the affected eye. The association be tween sickle cell trait and rheumatoid arthritis resulting in retinal vascular occlusive disease has not been reported previously. The prese nce of retinal vascular occlusion in sickle cell trait necessitates a medical evaluation for additional systemic diseases.