This feature is part of the Autoimmune Disease Awareness Month series.

When a child is diagnosed with a chronic autoimmune disease, parents may feel overwhelmed by the number of biological systems at risk. Although some will scramble to find appointments with various specialists, others may not even be aware of the importance of doing so. Who could blame them? After all, even many physicians are not fully apprised of the urgency of having various systems evaluated and managed as early as possible.1 This knowledge shortcoming appears to be of particular concern in the case of chronic uveitis associated with juvenile idiopathic arthritis (JIA). For this reason, the American College of Rheuma­tology (ACR) and the Arthritis Foundation convened a panel of ophthalmologists and other specialists to offer updated guidelines on the screening, monitoring, and treatment of JIA-associated uveitis.1

“There has been great variation in treatment practices, and it has been apparent to both uveitis specialists and pediatric rheumatologists that not all children are receiving optimum care. We often see pediatric patients who have been undertreated or whose treatment has been delayed,” Gary N. Holland, MD, one of the researchers and an ophthalmologist at UCLA Stein Eye Institute, told the American Academy of Ophthalmology.2


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The updated screening protocols are designed to unify the ophthalmic screening of children with a JIA diagnosis and help clinicians co-manage across specialties to stave off the most serious uveitis complication — vision loss — as long as possible.  

After Initial Diagnosis

Noninfectious uveitis that is not the result of trauma is quite rare in children, explains Marc Taub, OD, an educator and co-supervisor of the pediatric and vision therapy residency at Southern College of Optometry. But if a pediatric patient did present with such a case, “I would think rheumatoid arthritis,” he says.

Uveitis is the most common extra-articular manifestation of JIA.1 In fact, a 2020 study found this complication in 19.3% of participants, and it refers to previous research showing as many as  25% of JIA patients develop either chronic or acute uveitis.3 But the patient’s age and sex can help determine when, and in which patients, this comorbidity is likely to present. A study examining 109 patients with JIA shows that ocular involvement is most likely to present at 2 different peaks of age. The first is between the ages of 4 years and 6 years and the second is between 10 years and 12 years.1,3 These are the age groups in which most cases of JIA-associated uveitis are diagnosed.3 In a 2019 study, all the patients in the younger age group were girls and all the patients in the older age group were boys. In fact, female sex and younger age are established risk factors for uveitis in patients with JIA.3,4 Additionally, the younger group, the girls, presented with oligoarticular arthritis and chronic anterior uveitis whereas the second group, the boys, presented with different types of JIA and acute anterior uveitis. The overall prevalence of ocular complications was higher in the girls’ group.3 

The ACR has identified several factors that can put patients in the “high-risk” category. These factors include oligoarthritis, polyarthritis (rheumatoid factor negative), psoriatic arthritis, or undifferentiated arthritis who are also antinuclear antibody (ANA) positive. Patients younger than 7 years of age at JIA onset and who have a JIA duration of 4 years or less are also in this group. High-risk patients should be seen at least every 3 months.1 Low- or moderate-risk children are those with high-risk JIA categories but who are ANA negative, 7 years of age or older at JIA onset, or who have JIA duration of more than 4 years, and those with systemic JIA, polyarthritis (rheumatoid factor positive), and enthesitis-related arthritis. Low- or moderate-risk children should be screened every 6 months to 12 months.1,3

Chronic uveitis doesn’t always manifest visually, but even when it does children in the high-risk category may have trouble expressing some of their symptoms, Dr Taub cautions. These symptoms can include light sensitivities and eye pain. However, these are rare, especially in nonacute cases. Parents are unlikely to notice any ocular surface reddening and the children themselves do not frequently experience pain or any symptoms at all, making regular examinations all the more necessary.4

Treatments

Frequent monitoring of the patient’s intraocular pressure is vital, particularly in these patients, Dr Taub explains, as any uveitis can become glaucomatous. Not only can the inflammation instigate glaucomatous changes, but the glucocorticoid treatments used to manage the uveitis itself are associated with glaucoma as well as early cataract development.1,5,6 However, these complications are also associated with untreated disease.4 Additional complications can include band keratopathy, posterior synechiae, macular cystoid edema, hypotony, and epiretinal membrane formation, any of which can cause visual disturbances and, in some cases, vision loss.3

To avoid complications with topical glucocorticoids, the ACR recommends they should be used only as initial treatment to achieve control of inflammation. “Methotrexate and the monoclonal antibody tumor necrosis factor inhibitors adalimumab and infliximab are recommended when systemic treatment is needed for the management of uveitis,” the report adds. “The timely addition of nonbiologic and biologic drugs is recommended to maintain uveitis control in children who are at continued risk of vision loss.”1

Recommendations for Glucocorticoid Use

“Tapering can be an issue with these kids,” Dr Taub explains. “They could start to slide back if you do it too fast.”

In patients with JIA and active chronic anterior uveitis, the ACR researchers prefer using prednisolone acetate 1% topical drops to difluprednate topical drops. They also advise against adding systemic glucocorticoids. Instead, they suggest adding or increasing topical glucocorticoids for short-term control. However, in patients with JIA and chronic anterior uveitis who still require 1 drop to 2 drops prednisolone acetate 1% (or equivalent) daily for uveitis control, the ACR recommends adding systemic therapy to help taper topical glucocorticoids, if the patient is not already on systemic therapy.

Finally, they recommend using topical glucocorticoids prior to changing or escalating systemic therapy in favor of changing or escalating systemic therapy immediately in patients with JIA who develop new chronic anterior uveitis activity despite stable systemic therapy.

These steroid treatments could take months before patients are ready to begin tapering off.

Uveitis occurs infrequently in patients with JIA, but its potential for severe ocular complications, including vision loss, could be costly. Frequent ophthalmic evaluations for patients with JIA, whether or not they are being medically treated for uveitis, are vital to their visual and ocular health. The ACR’s guidelines advocate for close comanagement of patients with JIA between ophthalmology and rheumatology to deliver optimal care.1

References

1. Angeles-Han S, Ringold S, Beukelman T, et al. 2019 American College of Rheumatology/Arthritis Foundation guideline for the screening, monitoring, and treatment of juvenile idiopathic arthritis-associated uveitis. Arthritis Care Res. 2019;71(6):703-716. doi:10.1002/acr.23871

2. Roach L. New guidelines target uveitis in patients with JIA. EyeNet Magazine. Published online August 2019. 

3. Castagna I, Roszkowska A, Alessandrello F, et al. Juvenile idiopathic arthritis-associated uveitis: a retrospective analysis from a centre of South Italy. International Ophthalmol. Published online October 4, 2019. doi:10.1007/s10792-019-01184-8

4. Cleveland Clinic. Juvenile idiopathic arthritis and the eyes. Published online February 8, 2019. Accessed March 18, 2021. 

5. Kothari S, Foster CS, Pistilli M, et al. The risk of intraocular pressure elevation in pediatric noninfectious uveitis. Ophthalmol. 2015;122:1987–2001. doi:10.1016/j.ophtha.2015.06.041

6. Thorne JE, Woreta FA, Dunn JP, Jabs DA. Risk of cataract development among children with juvenile idiopathic arthritis-related uveitis treated with topical corticosteroids. Ophthalmol. 2010;117(7):1436-1441. doi:10.1016/j.ophtha.2009.12.003