The American College of Rheumatology (ACR) has issued two updated treatment guidelines for juvenile idiopathic arthritis (JIA). The recommendations reflect more effective medications, the importance of vaccination, and feedback from young adults with JIA, their caregivers, and providers.
“Changes within our field are occurring at an ever-increasing rate,” says Karen Brandt Onel, MD, the chief of the division of pediatric rheumatology at the Hospital for Special Surgery and a professor of clinical pediatrics at Weill Cornell Medicine in New York City, and the lead investigator for the new updates, released in early March 2022. “Our practice has dramatically changed in the past 10 years, and these guidelines have to change to reflect challenges and advances in our field.”
The guidelines recognize that every patient is different, has been diagnosed with a certain form of JIA, and that treatment decisions should be made in collaboration with the diagnosed person and their caregivers.
The ultimate goal is improved quality of life.
“The ability to change the outcome is empowering,” Dr. Onel says. “We want to be certain that every child is able to grow and thrive. For children with JIA, we hope that these guidelines will play an important role in making certain that all children with JIA can lead long, happy, healthy lives.”
What’s New in the Latest Recommendations for Treating Kids With Arthritis?
The new recommendations specify effective drug treatments for people living with oligoarthritis, temporomandibular joint (TMJ) arthritis, and systemic JIA (SJIA), and how the patient should stop taking them when they're no longer needed. The new guidelines are compatible with the 2019 ACR treatment recommendations for other forms of JIA.
People diagnosed with oligoarthritis (up to four joints affected) or TMJ arthritis (affecting jaw joints), should first consider a short treatment with nonsteroidal anti-inflammatory drugs (NSAIDs), or injected glucosteroid drugs. If these don’t quickly help with joint pain, swelling, and function, providers should prescribe disease-modifying antirheumatic drugs (DMARDs) — either conventional DMARDs that work to suppress the overall immune system, or biologic DMARDs that target specific parts of the immune process. Either way, such suppression can work to reduce or halt the joint inflammation and potential joint damage caused by JIA. “Conventional DMARDs are recommended as a first-line therapy for most types of JIA,” says Onel.
Weighing Health Risks and Health Benefits When Treating Children
Patients should work with their providers to determine what the best treatment plan is, says the patient-advocate Ashley Boynes-Shuck, a health coach and the author of two books about chronic illness, who has lived with JIA for 26 years. “I’m not a doctor, but I do think DMARDs can be an effective step for JIA patients before jumping to stronger or riskier drugs, as long as both the patient or caregiver and doctor are on board and well-versed in any potential adverse events, risks, or side effects,” she says. “You must always weigh risk versus benefit with any new treatment.”
Thanks to Advances, Young People With JIA Don’t Need to Rely on High-Dose Steroids
The ACR strongly recommends that people with systemic JIA (which affects joint function and may also cause a rash, high fevers, organ problems, joint damage, or anemia) should be initially treated with biologic DMARDs, instead of the high-dose steroids that were previously recommended but caused weakened bones, osteoporosis, and suppressed growth in those who took them long-term. Boynes-Shuck says she has experienced such side effects: “I have osteopenia [bone loss] in some of my bones because I was put on steroids at such a young age for my JIA.” She also experienced weight gain, mood swings, weakened bones, and an adrenal crisis, she says. Despite these and a number of other treatments that were not effective over the years, Boynes-Shuck says she does feel that "treatment options for JIA and rheumatoid arthritis have come a long way. And I am also hopeful about things in store for the future.”
Onel agrees. “Changes within our field are occurring at an ever-increasing rate,” she says. “Our practice has dramatically changed in the past 10 years, and these guidelines have to change to reflect challenges and advances in our field.
“Starting with a biologic can help avoid this, so the ability to minimize steroid use is a major achievement,” says Onel. “Previously, treating systemic JIA required the use of high-dose steroids, and many children suffered the consequences,” including the aforementioned bone and growth problems.
Medication innovations such as biologics are genetically engineered to work in a more targeted way. They differ from conventional treatments that are created chemically. The past 25 years have seen major advances in biologics that have helped improve the lives of people living with JIA, note researchers in a study published in 2020 in Rheumatology. In particular, biologics have helped reduce the need for orthopedic surgery and have lowered the rates of uveitis, a type of eye inflammation experienced by JIA patients.
Once the disease is considered inactive in those with systemic JIA, their providers should work with them to taper off the medications.
Nondrug Treatments Help JIA Too
The second set of ACR guidelines addresses nondrug treatments for those living with all forms of JIA. These include sessions of physical therapy (PT) and occupational therapy as needed; a “healthy, well-balanced, age-appropriate diet”; lab tests at the start of and during the course of medications to treat JIA; and being up-to-date on all recommended vaccinations. Since DMARDs affect the immune system, there are specific instructions for people whose immune systems are suppressed, as well as recommendations for those who are not on immunotherapy and who are not immunized. In short, all people with JIA and the people they live with should be fully vaccinated to prevent the transmission of illnesses such as COVID-19 and the flu.
“I firmly believe that preventive wellness measures and lifestyle changes are extremely important for being able to thrive with JIA — and just for overall health and well-being in general,” says Boynes-Shuck, who cited physical therapy (PT), occupational therapy (OT), psychotherapy and counseling, nutrition, sleep, immunizations, exercise, mindfulness, and a good support network as key components.
The updated ACR guidelines will help providers work with people living with JIA and their caregivers to live healthier lives with the condition, Onel says.
“There are real children and real families whose lives will be affected by our recommendations,” says Onel. “We very much hope that these dialogues will help us make a bright future for children with JIA.”