Clinical Images: Late‐stage hydroxychloroquine‐associated retinopathy

The patient, a 58‐year‐old woman with rheumatoid arthritis, presented with 6 months of central scotoma and photosensitivity. She had been treated with hydroxychloroquine at a dosage of 400 mg daily for 5 years, then 600 mg daily for another 5 years. Visual acuity was 20/25 and 20/30 in the right eye and left eye, respectively. A fundus examination and fundus autofluorescence revealed a characteristic “bull's‐eye” pattern of parafoveal pigment change in both eyes (representative left eye shown in color fundus photograph [A] and fundus autofluorescence image [B], with a dark hypofluorescent ring indicating atrophy of the retinal pigment epithelium; white arrows marking “bull's eye”). The retinal optical coherence tomography result was positive for the “flying saucer sign”—loss of the ellipsoid zone (orange arrow in C), representing photoreceptor damage in a ring pattern around the fovea (bracket in C). Visual fields revealed a paracentral scotoma in both eyes (D; central visual field overlaid to demonstrate field loss corresponding to the area of retinal pigment epithelium atrophy, with darker‐shaded areas of visual field corresponding to decreased sensitivity to light stimuli). A diagnosis of hydroxychloroquine‐associated retinopathy was made, and the medication was stopped. Retinal toxicity is a known and devastating side effect of  hydroxychloroquine due to increased cumulative and daily doses (1). This patient's 600‐mg daily dose exceeded the recommended real‐weight‐adjusted maximum dosage of 5 mg/kg/day. Her cumulative dosage of 1825 g also far exceeded the 1000‐g threshold that has been associated with increased risk of retinal toxicity (2). Although hydroxychloroquine‐associated retinopathy is irreversible, late effects, such as the bull's‐eye maculopathy seen here, are preventable. Through appropriate referral for baseline ophthalmologic examination, as well as annual screening starting at 5 years of therapy, toxicity can and must be detected early, avoiding presentations like this one (1).

Under the University of Michigan Human Research Protection Program Operations Manual Part 4 Section V, case studies including one or two patients are not subject to institutional review board oversight.