Diagnostic and Therapeutic Challenges

Abstract

Case ReportThis is a case of a 58-year-old Asian woman who returned for ophthalmic evaluation of pigmentary retinal changes and routine Plaquenil screening. She initially presented at age 57, without any visual complaints. She was being treated with Plaquenil 200 mg daily for a total of 5 years for systemic lupus erythematosus. On initial presentation, her vision in both eyes was 20/20 and anterior segment examination was unremarkable. Dilated fundus examination revealed a bilateral pigmentary change that encompassed both nerves and extended into the macula, with asymmetry, in which the left eye ( Figure 1, C and D) showed more macular involvement than did the right eye ( Figure 1, A and B). At that time, she was only observed. It was deemed safe for her to continue Plaquenil, given that at the time, her lifetime dosage was 389 g over 5 years, and daily dosage calculated based on ideal body weight was 4 mgÁkg −1 Áday −1 .At follow-up evaluation, a year later at age 58, she had no visual complaints. Her Plaquenil lifetime dosage had increased to 469 g. Visual acuity was 20/20 in both eyes, and the anterior segment examination remained unremarkable. The repeat dilated fundus examination exhibited progression of retinopathy in both eyes ( Figure 2). The extent of the lesions is best demonstrated using fundus autofluorescence (Figure 2, B and D). Spectral domain optical coherence tomography (Spectralis) images (Figure 3) revealed discrete irregularities at the level of the retinal pigmented epithelium, but without associated loss of photoreceptor segment lines. Fluorescein angiography (Figure 4) was consistent with a speckled and punctate pattern of hyperfluorescence in the area of the lesions. Interestingly, indocyanine green angiography ( Figure 5) also highlighted the affected retina but with a pattern of hypofluorescence as contrasted to fluorescein angiography. In addition, indocyanine green angiography also showed punctate hyperfluorescent lesions outside those that were clinically apparent, indicating more widespread involvement.Laboratory testing was obtained to rule out infectious and inflammatory conditions, including quantiFERON gold, FTA-ABS, and Lyme serology, which were negative. A recent PPD read negative as well.This patient is presented for diagnosis, prognosis, and management, and specifically whether to continue Plaquenil therapy.