Normalization of generalized retinal function and progression of maculopathy after cessation of therapy in a case of severe hydroxychloroquine retinopathy with 19 years follow-up

Abstract

A 40-year-old Caucasian female was first seen 20 years ago for a routine ocular screening in relation to hydroxychloroquine treatment for systemic lupus erythematosus. Her daily dose was 600 mg (or 12 mg/kg of body weight/day) of hydroxychloroquine. Three years later, she complained of mild visual loss in the right eye. Her best-corrected visual acuity was 0.9 in the right (RE) and 1.0 in the left eye (LE). In addition, she had a central scotoma (RE > LE) on automated visual field analysis (Humphrey central 30 degrees ). On fundoscopy and fluorescein angiography, the first signs of a bilateral bull's eye maculopathy were detected. A decreased Arden ratio on EOG (<1.50) was found with an accompanying decreased amplitude of the scotopic b-wave on full-field electroretinography in both eyes. Consequently, the treatment was immediately stopped. During the following years, the patient was retested regularly. After more than 18 years after cessation of the drug, most tests showed a further deterioration, including best-corrected visual acuity (RE: 0.1; LE: 0.7). On visual field testing, a progressive evolution to a total and absolute central scotoma in the RE (central 10 degrees ) and an annular scotoma in the LE became apparent. In contrast, a partial recovery of the Arden ratio of the EOG to 1.8 in both eyes was seen. In addition, a partial recovery of the scotopic b-wave full-field ERG was noted 19 years after cessation of treatment. At retest visits respectively 18, 19 and 20 years after cessation of hydroxychloroquine, a multifocal electroretinogram was performed in combination with colour vision tests and contrast sensitivity measurements.

Decreased Perifoveal Sensitivity Detected by Microperimetry in Patients Using Hydroxychloroquine and without Visual Field and Fundoscopic Anomalies

“…Nevertheless, there is high variability in the cumulative doses that lead to toxic retinopathy [ 11 ] and the damage may appear even in those patients with a low systemic risk profile. Also, a high risk of progression of the retinal damage has been suggested despite the cessation of the treatment [ 12 ] and a “point of no return” for macular toxicity [ 13 ]. Thus, an individual and weight-adapted dosing has been proposed to minimize the incidence of retinal damage [ 14 ].…”

Progression of Hydroxychloroquine Toxic Effects After Drug Therapy Cessation

“…3 Given the infrequent occurrence of toxic effects, few data are available on the presenting features and long-term follow-up of patients with hydroxychloroquine retinopathy, making it difficult to surmise the clinical course of patients after cessation of drug treatment. 6,[9][10][11][12][13] Yam and Kwok 5 speculated that toxic effects could be separated into premaculopathy and true retinopathy, with the opportunity for reversibility if changes are detected in the former stage. The most characteristic imaging features of hydroxychloroquine toxic effects include disruption of normal FAF, whereas on SD-OCT there is RPE atrophy and loss of the photoreceptor layer.…”

The Assessment of Structural and Functional Test Results for Early Detection of Hydroxychloroquine Macular Toxicity

“…photopic and mesopic conditions in a patient with SLE with HCQ retinopathy. The results of the patient were compared with 10 healthy people in the same age group and were found to be signi cantly lower than the healthy group at all spatial frequencies (24). Bishara et al reported high frequency of pathological and suspicious contrast sensitivity tests in the medication group (25).…”