This 28YO Indian female presented with several months of seeing occasional black spots. She has systemic lupus erythematosus, which has been treated with hydroxychloroquine (HCQ) for the past 5 years. Her calculated cumulative dose of HCQ was 365 grams. She denied having prior baseline or screening examinations for HCQ toxicity. Vision was 20/30 OU.
Color photography shows bilateral perifoveal pigmentary changes. Fundus autofluorescence (FAF) shows a rim of parafoveal hyper-FAF. OCT scanning shows paracentral outer retinal thinning, with disorganized clumps of hyperreflective material overlying thinned RPE.
Learning Points:
Current screening guidelines should make hydroxychloroquine toxicity, as found in our patient, a thing of the past. Last revised in 2016, the American Academy of Ophthalmology (AAO) recommends that a baseline exam be performed before starting the medication, with annual screenings beginning at least 5 years after initiating treatment, unless major risk factors are present. However, in practice, patients are usually screened yearly once they are placed on this medication.
OCT is performed annually to assess outer retinal findings, including ellipsoid zone loss. Humphrey 10-2 visual field testing (24-2 for Asians, since their macular involvement is usually more peripheral) is also needed annually, as about 10% of patients will have field loss despite normal examinations and OCT testing (Ahn et al., AJO 2017;184:11-18).
Plaquenil dosing should also be based on real, not ideal, weight to better predict the optimal dose. See Marmor et al Ophthalmology 2016;123:1386-1394 for the full screening guidelines. Despite the most recent AAO guidelines being almost 10 years old, a recent publication found that over one-third of patients are still overdosed (Moussa et al, Ophthalmology Retina 2025;9:814-817).
Toxicity still developed in our patient despite not falling into any of the high-risk categories (cumulative HCQ dose < 1000 grams, young age, not obese). We have written a letter to her rheumatologist to immediately stop HCQ treatment.
