Target in Sight: A Comprehensive Review of Hydroxychloroquine-Induced Bull's Eye Maculopathy

Abstract

Purpose of review: We review the latest screening and diagnostic techniques, and the most recent recommendations on the management of hydroxychloroquine retinopathy.

Recent findings: Hydroxychloroquine (HCQ) has been shown to cause retinal toxicity in a dose-dependent fashion. Early diagnosis is critical as the resultant retinopathy is not reversible. New imaging modalities, such as adaptive optics (AO), microperimetry, and retro-mode imaging, may show promise in the timely diagnosis of HCQ retinopathy.

Summary: Automated visual fields and spectral-domain optical coherence tomography (SD-OCT) are the primary tests used in routine screening for HCQ retinopathy, but fundus autofluorescence (FAF) and multifocal electroretinogram (mfERG) have also been shown to be useful. A baseline ophthalmologic examination is recommended in all patients beginning long-term hydroxychloroquine therapy within the first year of starting therapy. Automated visual fields and SD-OCT should be included during this baseline exam in patients with pre-existing macular conditions. Afterwards, annual screening can be deferred for the first 5 years of HCQ treatment unless the patient has a major risk factor.

Keywords: Bull’s Eye maculopathy; Bull’s Eye retinopathy; Hydroxychloroquine retinopathy; Hydroxychloroquine toxicity; Plaquenil retinopathy; Plaquenil toxicity.

Figure 1.

A. Color fundus photo of the left eye demonstrating “Bull’s Eye” maculopathy. Paracentral depigmentation of the retinal pigment epithelium (RPE) that spares the central fovea can be visualized. B. Fundus autofluorescence (FAF) photo of the left eye showing parafoveal hypo autofluorescence due to the low level RPE metabolic activity with underlying local atrophy with secondary photoreceptor loss. C. Optical coherence tomography (OCT) of the macula showing significant loss of the parafoveal photoreceptor inner segment/outer segment (IS/OS) junction and thinning of the outer retina with RPE irregularity. D. Humphrey visual field central 10–2 white-on-white pattern demonstrates dense paracentral scotoma.