0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Original Investigation |

Effect of Disease Stage on Progression of Hydroxychloroquine Retinopathy

Michael F. Marmor, MD1; Julia Hu1
[+] Author Affiliations
1Department of Ophthalmology, Byers Eye Institute, Stanford University School of Medicine, Palo Alto, California
JAMA Ophthalmol. 2014;132(9):1105-1112. doi:10.1001/jamaophthalmol.2014.1099.
Text Size: A A A
Published online

Importance  Hydroxychloroquine sulfate retinopathy can progress after the drug is stopped. It is not clear how this relates to the stage of retinopathy or whether early screening with modern imaging technology can prevent progression and visual loss.

Objective  To determine the relationship between progression of retinopathy and the severity of disease using objective data from optical coherence tomography and assess the value of early screening for the toxic effects of hydroxychloroquine.

Design, Setting, and Participants  Clinical findings in patients with hydroxychloroquine retinopathy were monitored with repeated anatomical and functional examinations for 13 to 40 months after the drug was stopped in a referral practice in a university medical center. Eleven patients participated, with the severity of toxic effects categorized as early (patchy parafoveal damage shown on field or objective testing), moderate (a 50%-100% parafoveal ring of optical coherence tomography thinning but intact retinal pigment epithelium), and severe (visible bull’s-eye damage).

Main Outcomes and Measures  Visual acuity, white 10-2 visual field pattern density plots, fundus autofluorescence, spectral-density optical coherence tomography cross sections, thickness (from cube diagrams), and ellipsoid zone length.

Results  Visual acuity and visual fields showed no consistent change. Fundus autofluorescence showed little or no change except in severe cases in which the bull’s-eye damage expanded progressively. Optical coherence tomography cross sections showed little visible change in early and moderate cases but progressive foveal thinning (approximately 7 μm/y) and loss of ellipsoid zone (in the range of 100 μm/y) in severe cases, which was confirmed by quantitative measurements. The measurements also showed some foveal thinning (approximately 4 μm/y) and deepening of parafoveal loss in moderate cases, but the breadth of the ellipsoid zone remained constant in both early and moderate cases. A few cases showed a suggestion of ellipsoid zone improvement.

Conclusions and Relevance  Patients with hydroxychloroquine retinopathy involving the retinal pigment epithelium demonstrated progressive damage on optical coherence tomography for at least 3 years after the drug was discontinued, including loss of foveal thickness and cone structure. Cases recognized before retinal pigment epithelium damage retained foveal architecture with little retinal thinning. Early recognition of hydroxychloroquine toxic effects before any fundus changes are visible, using visual fields and optical coherence tomography (along with fundus autofluorescence and multifocal electroretinography as indicated), will greatly minimize late progression and the risk of visual loss.

Figures in this Article

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

First Page Preview

View Large
First page PDF preview

Figures

Place holder to copy figure label and caption
Figure 1.
Variation in Pattern Deviation Plots After Hydroxychloroquine Is Discontinued

Repeated fields from earliest to latest measurement are shown from representative early (E), moderate (M), and severe (S) cases. The numbers at the upper left of the images indicate the months since hydroxychloroquine was discontinued, and the lower right number represents the pattern SD values. OS indicates left eye.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.
Fundus Autofluorescence Images After Hydroxychloroquine Is Discontinued

The images are from representative cases taken at near-onset (left column) and at the end (right column) of observation. The numbers at the upper left of the images indicate the months since hydroxychloroquine was discontinued. OD indicates right eye; OS, left eye.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3.
Spectral Density–Optical Coherence Tomography Cross Sections After Hydroxychloroquine Is Discontinued

The images are from representative cases at near-onset (left column) and at the end (right column) of observation. The numbers at the upper left of the images indicate the months since hydroxychloroquine was discontinued. OD indicates right eye; OS, left eye.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 4.
Change in Foveal and Parafoveal Thickness After Hydroxychloroquine Is Discontinued

The graphs show change from baseline near the time hydroxychloroquine was discontinued. Mean values were determined using data from the 2 eyes; parafoveal values are the mean of the temporal, superior, nasal, and inferior regions. OCT indicates optical coherence tomography.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 5.
Change in Foveal Ellipsoid Zone After Hydroxychloroquine Is Discontinued

The graphs show change from baseline near the time hydroxychloroquine was discontinued. Mean values were determined using data from the 2 eyes; results are shown for vertical and horizontal slices (cross sections).

Graphic Jump Location

Tables

References

Correspondence

CME


You need to register in order to view this quiz.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles
Jobs
brightcove.createExperiences();