Non-leaking cystoid macular edema and bull’s eye maculopathy caused by hydroxychloroquine toxicity in Asian patients

Abstract

Introduction: Hydroxychloroquine (HCQ) is used for treating systemic lupus erythematosus (SLE). It can cause irreversible toxic retinopathy, we discuss the outcome of HCQ retinopathy and emphasize the distinct toxicity pattern in Asian patients. Case Series: We report a retrospective case series of two systemic lupus erythematosus (SLE) patients who presented with HCQ toxicity. Both Asian SLE patients were treated with HCQ over five years with cumulative dose of >1000 g. Both had characteristic findings on spectral domain optical coherence tomography (SD-OCT) and fundus autofluorescence (FAF). First patient had non-leaking cystoid macula edema (CME) associated with HCQ retinopathy and second patient had bull’s eye maculopathy (BEM). They exhibited different outcome after drug cessation. There was no disease progression in former whereas latter lost her central vision over two years. Conclusion: Non-leaking CME is rare and can be a sequence of HCQ retinopathy. Bull’s eye maculopathy is another manifestation of severe HCQ toxicity. Toxic damage to retina is irreversible, and may progress even after the drug is stopped, so is crucial to discontinue once toxicity is detected. Patients on HCQ warrant annual screening with multimodal imaging. There are racial differences in HCQ toxicity, hence distinct screening tests should be performed in Asian population.