When your toddler is diagnosed with juvenile idiopathic arthritis, and your mind fills with worrisome visions of those small, supple joints growing progressively more swollen, stiff and painful, the last thing you’re probably thinking is that your child could go blind.
But she or he could, and it could happen with few warning signs.
Uveitis, an often-silent inflammation of the eye, is a serious side effect of juvenile idiopathic arthritis, an umbrella term that encompasses seven different forms of inflammatory, autoimmune rheumatic diseases that can affect teens and children 16 years of age and younger.
According to pediatric rheumatologist Dr. Melissa Lerman, “approximately 15 percent of patients with JIA will develop uveitis.”
Uveitis is even more prevalent in cases of oligoarticular JIA, which is the most common, and usually the mildest, form of JIA. Twenty-five percent of children with oligoarticular JIA develop uveitis, and of these, about 12 percent go blind, usually as a result of chronic, low grade inflammation.
Girls have a higher risk of developing uveitis than boys, as do children younger than three years of age when diagnosed with JIA. Also, children with JIA have a greater chance of getting uveitis if they test positive for a protein called an anti-nuclear antibody. Antibodies are produced to fight off foreign proteins that lead to infection. When the antibodies go after naturally occurring proteins, they’re called autoantibodies, and the ones that target normal proteins within the nucleus of a cell are called anti-nuclear antibodies.
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If not diagnosed and treated properly, uveitis can cause permanent vision loss.
“Children and adolescents with JIA-associated uveitis should have regular screening by an ophthalmologist even though they do not have vision changes,” advises Lerman, who is an assistant professor of clinical pediatrics in the Division of Rheumatology at the Perelman School of Medicine at the University of Pennsylvania.
Pediatric rheumatologist Dr. Sheila T. Angeles-Han echoes these sentiments. “It is crucial that children at high risk are evaluated by a pediatric ophthalmologist every three months, especially since children with uveitis usually do not have any eye symptoms,” says Angeles-Han, who is an assistant professor of pediatrics in the Division of Rheumatology at the University of Cincinnati Children’s Hospital Medical Center.
“The frequency of ophthalmology visits will vary, depending on the child’s risk factors,” Angeles-Han says.
“The most critical aspect of JIA-associated uveitis is that patients can have severe inflammation but not have any symptoms until significant vision loss has occurred,” says Dr. Stefanie Davidson, attending surgeon in the Division of Pediatric Opthalmology at Children’s Hospital of Philadelphia. “Typical symptoms of uveitis include eye redness, pain, light sensitivity and blurred vision,” she says, adding that “these symptoms are often absent in JIA patients.”
“Vision loss can be related to cataracts, glaucoma, macular edema [swelling of the macula, an area in the center of the retina], and retinal complications, including neovascularization [overgrowth of blood vessels in the retina],” Davidson explains. “Other complications include synechiae (scarring) in the front part of eye. Uveitis associated with JIA typically only affects the front part of the eye.”
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Steroid eye drops are the first line of defense against uveitis to reduce inflammation of the eye. “However, long-term use of steroids can cause cataracts and glaucoma,” Davidson says. “Therefore, when uveitis is chronic, as is the case with patients with JIA, steroid-sparing systemic agents are prescribed, including methotrexate and [biologics] like infliximab and adalimumab.
“Children should receive frequent (up to weekly) eye exams to ensure that uveitis is controlled and to ensure that it remains controlled as the topical steroids are tapered,” Angeles-Han advises.
It’s important for parents to understand that uveitis is a chronic disease, Lerman says. “Patients with JIA-associated uveitis may require long-term treatment with systemic agents, which are most often injectable drugs.”
“Children [typically] should remain on these medications for one to two years of ‘quiet’ uveitis, once they are off all topical and systemic steroids before any attempt at weaning should be made,” she says. “Some children are able to discontinue uveitis medications; others are not able to wean off of them without having uveitis recur. Sometimes this happens early, but sometimes it occurs a while afterwards.”
Also, “uveitis and arthritis do not follow the same course,” she says. This means that uveitis can still be present even when JIA has gone into remission.
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“Once a patient has been diagnosed with uveitis and is in treatment, parents cannot independently decide that the patient is doing well and [has been] treated adequately,” Angeles-Han say. “Medication must be continued as prescribed, and should only be tapered or discontinued as directed by an ophthalmologist,” she says.
It’s not all bad news, however.
“With appropriate vision screening and close follow-up, we expect children to have very good visual outcomes,” Lerman says.
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Juvenile Arthritis May Cause Blindness originally appeared on usnews.com
