May. 11, 2014

Vision Quest

By Jenny Thorn Palter

With all of the physical and emotional complications that lupus can cause, it’s easy to overlook symptoms that seem minor at first. But take this advice: Don’t ignore changes in vision.

While side effects of corticosteroids are usually to blame, there is also the possibility of ocular damage associated with long-term use of the antimalarial drugs hydroxychloroquine (brand name: Plaquenil®) and chloroquine. Improved vision screening tests and updated dosing guidelines are key to avoiding such complications.

On the lookout

Ophthalmologist and medical retina specialist Jonathan Lyons, M.D., who practices in Silver Spring, Md., and teaches at MedStar Washington Hospital Center and MedStar Georgetown University Hospital, both in Washington, D.C., has long been involved in developing the standards for what ophthalmologists should look for during vision screenings.

“The multifocal ERG technology (mfERG) was developed in the ’90s. With this, we are able to look at specific areas of the retina. Prior to this, we weren’t able to do that in an objective manner,” he says. “I’ve developed a method of looking at these tests to see which part of that pattern is abnormal in patients who have toxicity. The toxicity is confirmed by using the visual field, asking the patient for specific symptoms, and then using the mfERG to confirm the diagnosis at the earliest possible moment.”

Lyons emphasizes the importance of annual screening and says that, although annual screening is recommended for everyone taking Plaquenil, it is imperative for people who have been taking the medication for more than 10 years, who have a higher incidence of retinal toxicity.

“Eye damage due to Plaquenil is not common,” he says. “Rarely will anybody who has good vision and minimal symptoms develop loss of central vision or ability to read if annual screening is done and visual symptoms reported as soon as they occur so the medication can be stopped if toxicity occurs.”

He adds, “In doing those tests once a year we should be able to prevent progression of retinal damage.”

Dosage decisions

Donald E. Thomas Jr., M.D., F.A.C.R., F.A.C.P., a practicing rheumatologist in Greenbelt, Md., and author of The Lupus Encyclopedia, regularly prescribes Plaquenil to his lupus patients. “We know that people with lupus who take their Plaquenil regularly are much more likely to live a long, normal life compared with those who don’t,” he says. “They also are less likely to develop major organ involvement.”

Thomas says he understands people’s concerns about the drug’s potential for toxicity. “But you can’t even compare it to the only other things I have [to treat lupus]—prednisone, where 100 percent of people get side effects, or immunosuppressive drugs, [which] suppress the immune system, potentially causing infections, low blood counts, and liver toxicity. We just need to get better at monitoring the eyes and dosing Plaquenil correctly.”

New dosing guidelines for Plaquenil, which Lyons worked on, were published in February 2011 in the journal Ophthalmology. (See sidebar.) Thomas is enthusiastic about the updated guidelines, although he worries that the new dosing recommendations have not been widely disseminated to rheumatologists. “I think people with lupus can make a difference by pointing out the new guidelines to their rheumatologists,” he says.

Changes in vision

Ann Utterback, 67, a broadcast voice specialist who lives in Gaithersburg, Md., was diagnosed with lupus in 2005. She says she’s always been extra-sensitive to medications, and her vision loss is related to both corticosteroids and Plaquenil.

The first thing that happened was I couldn’t read my digital clock, because the bars of light were broken,” she recalls. “That was in early 2011. Then I noticed I could not see well in a scintillating, or shimmering, donut-shaped area of my right eye.” She had the same problem in her left eye, although to a lesser extent.

Her ophthalmologist detected a cataract in her right eye, caused by steroids, but he didn’t think it was causing the problem she was having. After cataract surgery she noticed a difference, but it was not what she was expecting.

“After the surgery, the blind area was more noticeable because my sight in that eye was so much clearer,” she says. “The surgeon sent me to a retina specialist, who recommended that I cut down my dose of Plaquenil. Finally, my rheumatologist recommended that I stop taking the drug altogether.”

Utterback’s rheumatologist also referred her to neuro-ophthalmologist David Katz, M.D., in Bethesda, Md. “We don’t know which patients might develop Plaquenil retinopathy, but the patient often can see it before the doctor can,” he says.

Katz stresses that there are some important warning signs. “If you feel like your central vision is getting blurred, if you lose the ability to see a digital clock, if you lose color vision—if reds look kind of washed out—or if you have trouble seeing at night: Those are early signs that you might be developing Plaquenil retinopathy,” he says.

There are guidelines on the amount of Plaquenil a person should be able to take safely in a lifetime, and Katz encourages those taking the medication to discuss this with their ophthalmologists. “I think there are some people, like Ann, whose bodies, for whatever reason, don’t metabolize drugs as well,” he says. “Maybe someday we’ll have a blood test to determine who’s at high risk.”

According to Lyons’ research, prevalence of toxicity after five to seven years of Plaquenil use approaches 1 percent of patients, although the risk after 15–20 years of exposure is higher. 

Still, “it is not necessary to discontinue the medication regardless of cumulative dose if careful, regular screening is done,” Lyons says. “We can detect changes early enough to prevent any significant effect on vision, and stopping the medication at that point appears to halt progression of the changes.”

Words of wisdom

“The message I want to get out is: Pay a lot more attention to side effects of medication and any changes in vision,” Utterback says. “I never in a million years thought anything would happen to my eyes, but it does happen.”

To help preserve vision over the long term, know the recommendations. “Today it is recommended that every single person on Plaquenil get a visual field 10-2 test, plus one of the three other highly sensitive screening tests: the FAF (fundus autofluorescence imaging), the SD-OCT (spectral domain-ocular computerized tomography), or the multifocal electroretinogram (mfERG),” Thomas says. “A lot of times, the rheumatologist will just write a note to the ophthalmologist, saying, ‘Do screening tests once a year.’ But really, the rheumatologist should be writing, ‘Do a visual field 10-2, plus one of these three advanced screening tests.’ ”

If you have lupus, ask your rheumatologist for a referral to an ophthalmologist who has up-to-date technology to evaluate your vision for any early problems. Whether the medication is an antimalarial or a corticosteroid, one thing is clear: Today’s new screening methods, coupled with vigilance on the part of the patient, will go a long way to ensuring that vision damage and loss are problems of the past.

Read the abstract of “Revised recommendations on screening for chloroquine and hydroxychloroquine retinopathy.” Ophthalmology 2011 Feb; 118(2):415–22 at

How to Dose Plaquenil

“Plaquenil (hydroxychloroquine) is not deposited into fat,” explains Donald E. Thomas, Jr., M.D., F.A.C.R., F.A.C.P. “That is why it is recommended that we go by ideal body weight, or IBW—taking into account lean body mass more so than any extra fat mass in people who are overweight.”

Thomas explains that the American Association of Ophthalmology recommends using the “Method of Devine” to determine IBW: For women, start with 100 pounds, and add 5 pounds for every inch over 5 feet. For men, start with 106 pounds, and add 6 pounds for every inch over 5 feet. The maximum dose of Plaquenil is 6.5 mg/kg IBW/day, regardless of whether a person is overweight, underweight, or normal weight. One kilogram (kg) equals 2.2 pounds.

Women with lupus who are over their IBW should go by these dosing rules:

5’ 7” or higher - up to 400 mg Plaquenil daily

5’ 1/2” to 5’ 6 1/2” - up to 300 mg Plaquenil daily

5’ or shorter - up to 200 mg Plaquenil daily

Women with lupus who are at or below their IBW should go by these dosing rules: 

135 lbs. or  more, any height - up to 400 mg Plaquenil daily

102 lbs. to 134 lbs. - up to 300 mg Plaquenil daily

101 lbs or less - up to 200 mg Plaquenil daily

From The Lupus Encyclopedia: A Comprehensive Guide for Patients and Families, by Donald E. Thomas, published by Johns Hopkins University Press. Reprinted by permission of the publisher.


Learn more about the Amsler grid. Although it is very nonspecific and not appropriate for screening in the doctor’s office, you can use it at home to help detect early problems.

Find out how lupus can affect your eyes. Systemic lupus is a chronic autoimmune disease that can affect any part of the body, including the eyes. 

The Truth About Toxicity

Ophthalmologist and medical retina specialist Jonathan Lyons, M.D., points out that Plaquenil-induced toxicity usually will not occur before five years of taking the drug.

Lyons’ advice to patients: “The earliest true signs would be kind of a shimmering light, so-called ‘photopsias,’ which often are in a circular pattern. Any change in vision—whatever you perceive as different from normal—should be checked out.”

Lyons’ advice to rheumatologists: “When taking patient histories, ask about symptoms affecting their vision. Encourage them to talk about any changes.”

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