NEW YORK – Tailoring the frequency of vision screening for children with juvenile idiopathic arthritis is an important step to getting uveitis symptoms treated effectively before permanent morbidity occurs, according to Dr. Sanjay R. Kedhar.
"We know that children with JIA [juvenile idiopathic arthritis] who have chronic inflammation of the eye have a threefold increase in the risk of blindness. We also know that immunosuppressive therapy reduces the risk of blindness by 60%. I believe that physicians should feel the time pressure to identify uveitis in children and treat them aggressively to preserve vision and reduce morbidity," said Dr. Kedhar, an ophthalmologist at The New York (N.Y.) Eye and Ear Infirmary and associate professor of ophthalmology at New York Medical College in Valhalla, N.Y. Uveitis-associated morbidity includes cataracts, glaucoma, band keratopathy, phthisis bulbi, and vision loss.
Dr. Sanjay R. Kedhar
Uveitis is the third most common cause of blindness in children, and JIA-associated uveitis is the most common cause of uveitis in children. About one in four children with JIA uveitis presents with blindness. Early detection, awareness of risk factors, and prompt and effective treatment can slow or prevent vision loss in children with JIA-associated uveitis, he said at the meeting, sponsored by New York University.
Uveitis associated with JIA most commonly affects the anterior portions of the eye, between the cornea and iris. Symptoms of anterior uveitis include blurred vision, pain, redness, photophobia, floaters, flashing lights, and distorted vision (metamorphopsia). The problem is that symptoms may be absent in children or, alternatively, children may not be able to verbalize what they see. "By the time symptoms are detected in school screening, it may be too late," Dr. Kedhar said.
That is why it is important to make sure children with JIA have regular ophthalmologic examinations as recommended by the American Academy of Pediatrics (Pediatrics 2006;117:1843-5). Screening frequency depends upon the presence of risk factors, such as type of arthritis (oligoarticular), age at onset, antinuclear antibody (ANA) seropositivity, and RF seronegativity. Children with JIA who have oligoarticular and polyarticular joint involvement and who are ANA positive and RF negative generally are at highest risk of developing uveitis, with 33%-70% affected.
"These are the kids we have to worry about the most," Dr. Kedhar said. If the joint symptoms appear before 7 years of age, the children should be seen every 3-4 months. Those children who have oligoarticular and polyarticular joint involvement beginning before age 7 but are ANA negative are considered at intermediate risk and should be seen every 6 months. Those who have a systemic onset of disease require screening every 12 months, reflecting the low risk of uveitis in this group.
Other risk factors for JIA-associated uveitis vision loss include female sex, anterior chamber flare seen on first exam, and abnormal intraocular pressure. The Systemic Immunosuppressive Therapy for Eye Diseases Study, a multicenter, retrospective cohort study of 327 patients with JIA-associated uveitis, showed that posterior synechiae, active uveitis, and intraocular surgery were associated with worse visual acuity outcomes (Ophthalmology 2013;120:186-92).
Another predictor of poor visual outcome is the onset of ocular symptoms before or soon after arthritis symptoms. Generally, having one ocular complication increases the risk of developing another.
Dr. Kedhar strongly suggested having an ophthalmologist be a part of the JIA patient’s medical team, along with the pediatrician and pediatric rheumatologist. A characteristic sign of anterior uveitis is an abnormal, irregular pupil. Ciliary flush is often not present in cases of JIA. In the acute setting, the red reflex may be diminished in the uveitis-affected eye. Keratic precipitates are white blood cells that accumulate on the posterior corneal surface and may indicate long-standing inflammation. Flare is another classic finding, and can be attributed to protein leakage into the aqueous humor. "In some studies, flare is used to monitor response of uveitis to treatment," Dr. Kedhar said. Uveitis is not typically associated with discharge or itching.
While topical corticosteroids are considered the first line of treatment, their use generated quite a bit of discussion from audience members. One of the main concerns is the conundrum that cataracts and glaucoma can occur because of uncontrolled inflammation associated with uveitis or as side effects of topical corticosteroids. Dr. Kedhar said that in his practice, he tries to limit the chronic use of corticosteroid drops to four times a day or less, generally switching patients to immunomodulatory therapy if they are corticosteroid dependent. He says that side effects are related to cumulative dose. He acknowledges that some ophthalmologists avoid topical steroids altogether but he believes they can be used safely for limited times. Because of greater concern for systemic side effects in children, systemic corticosteroids are used for only the short term.