Rapidly Progressive Hydroxychloroquine Retinal Toxicity Presenting With Cystoid Macular Edema and Retinal Vasculopathy: A Novel Phenotype With 6-Year Follow-Up.

PURPOSE: To report a unique case of hydroxychloroquine-associated retinal toxicity presenting with cystoid macular edema (CME) and retinal vasculopathy and to discuss its potential pathophysiology.

METHODS

A single case was reviewed.

CASE REPORT: A 65-year-old woman with a history of hydroxychloroquine use for approximately 5.5 years presented with bilateral CME and parafoveal ellipsoid zone disruption with outer retinal loss on optical coherence tomography. Multimodal imaging demonstrated central bull's-eye maculopathy and peripheral pigmentary changes on fundus autofluorescence and peripheral vascular leakage on fluorescein angiography. The uveitis workup, anti-retinal antibody testing, and genetic testing were negative. As retinal findings were normal at the first hydroxychloroquine screening, the patient was diagnosed with advanced hydroxychloroquine toxicity.

CONCLUSIONS

This case expands the phenotypic spectrum of hydroxychloroquine retinal toxicity to include CME associated with retinal vasculopathy. Annual retinal examination should begin promptly in patients receiving hydroxychloroquine at doses exceeding 5 mg/kg/day.