Purpose: To report an intractable case of retinal vasculopathy associated with mixed connective tissue disease (MCTD).
Design: Case report.
Methods: Interventional case report.
Results: A 35-year-old woman presented with decreased visual acuity and multiple retinal hemorrhages and macular edema in her right eye. She had a history of central retinal vein occlusion in the opposite eye and MCTD. Her clinical course deteriorated despite subsequent systemic and intravitreal steroid and immunosuppressant treatments. The retinal vasculopathy involved both artery and vein, causing wide retinal areas of nonperfusion. Her final visual acuity was hand motion.
Conclusions: MCTD may be associated with bilateral vasculopathy that is refractory to systemic steroid, intravitreal steroid, and immunosuppressant treatment.