New guidelines were released by the American College of Rheumatology (ACR) and the Arthritis Foundation (AF) to provide orientation to doctors and patients for the treatment of juvenile idiopathic arthritis (JIA).
Key recommendations addressing JIA-related non-systemic polyarthritis, sacroiliitis, enthesitis, and uveitis were compiled by a panel of pediatric rheumatologists, ophthalmologists, methodologists, adult patients, and parents of children affected by the disease.
The two guidelines, “2019 American College of Rheumatology/Arthritis Foundation Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Non-Systemic Polyarthritis, Sacroiliitis, and Enthesitis” and “2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis–Associated Uveitis,” were published in the journal Arthritis Care & Research.
The term JIA is used to describe several rheumatic autoimmune and inflammatory conditions, all of which involve chronic (long-term) joint inflammation, that develop before a patient turns 16. The disease may involve one or many joints, and it also may manifest with other symptoms, such as fevers or rash, as well as by a form of eye inflammation called uveitis.
“A number of treatments are available, including nonsteroidal anti-inflammatory drugs (NSAIDs), systemic and intraarticular glucocorticoids, and non-biologic and biologic disease-modifying antirheumatic drugs (DMARDs). Prompt initiation of appropriate therapy is of critical importance in preventing permanent damage and improving outcomes,” the panel wrote in one of the guidelines.
While earlier diagnosis and expanded treatment options have improved disease control, “they have also made the decision-making regarding treatments more complex for physicians, caregivers, and patients,” they added.
For JIA polyarthritis, the panel strongly favors initial therapy with DMARDs over NSAIDs, given as a single therapy. NSAIDs or intraarticular glucocorticoids are conditionally recommended as an adjunct (add-on) treatment, based on the very low quality of evidence supporting its use along with patient and caregiver preferences, and concerns regarding adverse side effects.
Starting treatment with a combination of a biologic therapies, such as etanercept (sold as Enbrel, and others), adalimumab (Humira, Cyltezo, among others), golimumab (Simponi), abatacept (Orencia), or tocilizumab (Actemra), and a DMARD also is conditionally recommended over the use of biologic agents alone.
The panel strongly recommended against adding low-term, low-dose glucocorticoids, irrespective of risk factors or disease activity. This decision was supported by the known adverse effects of this regimen in children, particularly growth suppression, weight gain, loss of bone mass, and cataracts (clouding of the eye’s lens).
For young patients at risk for functional limitations, it is recommended to get physical therapy and/or occupational therapy. This recommendation is conditional considering the low quality of evidence supporting a benefit for such interventions and based on the panel’s experiences.
“These recommendations highlight the importance of prompt and effective treatment for children with JIA and polyarthritis, sacroiliitis, and enthesitis,” Sarah Ringold, MD, MS, a pediatric rheumatologist at Seattle Children’s Hospital and the principal investigator on this guideline, said in a press release.
“They also support relatively tight disease control, with inactive disease as the goal. While it is anticipated that these recommendations will lead to improved outcomes for children with JIA and these phenotypes, they also emphasize the ongoing need to generate high-quality data about treatment effectiveness in JIA,” she said.
The second guideline released important recommendations for the management of JIA-associated uveitis. Chronic inflammation of the eye, medically identified as chronic anterior uveitis (CAU), affects about 10-20% of JIA children, and acute anterior uveitis (AAU) typically occurs in children with spondyloarthritis — meaning those with enthesitis or psoriatic arthritis.
For this group of patients the panel strongly recommended a more frequent ophthalmologic monitoring, particularly for those with controlled uveitis. This is specifically important to be done within one month after each change of topical (applied directly) glucocorticoids, within two months for those who are tapering or discontinuing systemic therapy (non-biologic DMARDs and biologic therapies), and at least every three months for patients on stable therapy.
For children and adolescents with severe, active CAU and sight-threatening complications, there is a conditional recommendation to immediately start methotrexate (a DMARD agent) plus one of the available anti-TNF biologics — infliximab (sold as Remicade, Flixabi, among others) or adalimumab —rather than methotrexate alone. This recommendation was conditional given the lack of direct evidence from clinical studies, the risk of permanent vision loss, and anticipated differences in patient values and preferences.
In addition, the guidelines strongly recommend educating JIA patients with spondyloarthritis regarding the warning signs of AAU so that it is possible to reduce delays in treatment, duration of symptoms, or complications of iritis (inflammation of the colored structure of the eye).
“Prevention of sight-threatening complications from uveitis is most important. It is crucial that children with JIA undergo scheduled ophthalmology screening to detect uveitis early since children are usually asymptomatic,” said Sheila T. Angeles-Han, MD, a rheumatologist at the Cincinnati Children’s Hospital and principal investigator for this guideline.