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.

What your eye doctor will look for

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

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. Plaquenil-induced toxicity usually will not occur before five years of taking the drug.

“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 are symptoms reported as soon as they occur so the medication can be stopped if toxicity occurs. In doing those tests once a year we should be able to prevent progression of retinal damage.”

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.”

New Plaquenil dosing guidelines increase safety

Donald E. Thomas Jr., MD, FACR, FACP, 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 the journal Ophthalmology. 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.

“Plaquenil is not deposited into fat,” he explains. “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.”

The American Association of Ophthalmology recommends using the "Devine formula" to determine ideal body weight. 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 of ideal body weight per 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 ideal body weight 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 ideal body weight 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

A lupus patient explains her 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, MD, 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.

Here 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,” says Katz.

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.”

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.”

Which annual screening tests to ask for

“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 optical coherence), 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.

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

Ideal body weight Plaquenil dosing calculations are reprinted with permission from The Lupus Encyclopedia: A Comprehensive Guide for Patients and Families, by Donald E. Thomas, published by Johns Hopkins University Press.