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Systemic Juvenile Idiopathic Arthritis Treatment

Systemic Juvenile Idiopathic Arthritis Treatment
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More recent medications have expanded treatment options for kids with Systemic Juvenile Idiopathic Arthritis (SJIA). 

The last few years have seen a revolution in treatment for SJIA. The introduction of targeted medications for SJIA has contributed to a change in the way many specialists approach therapy as well as its goals. Traditionally, a typical treatment goal for children with SJIA included relieving pain and controlling symptoms. But now, physicians also aspire to achieve disease remission with the ultimate hope of seeing the clinical signs and symptoms of the disease disappear.

SJIA Treatment Options

The standard approach to treating SJIA is to start with nonsteroidal anti-inflammatory drugs (NSAIDs) and move up to stronger drugs over time, if necessary. If NSAIDs don't relieve SJIA symptoms within a week, the next step may include high doses of oral or IV corticosteroids, which can quickly improve fever and other systemic symptoms, as well as joint pain and swelling.

But long-term, high-dose use of corticosteroids can cause serious side effects in children, including osteoporosis, slowed growth, an increased risk of infections, weight gain, and a change in facial appearance. When SJIA symptoms are under control, doctors gradually reduce the dose and eventually stop it completely. If symptoms return or a child can't tolerate corticosteroids, a different medication is often used.

Until recently, however, few treatment alternatives to corticosteroids existed for kids with SJIA. But over the past few years, the FDA has approved medications that target specific inflammatory proteins, including interleukin-1(IL-1) and interleukin-6 (IL-6), which are overactive in the disease.

These drugs, known as biologics, were once used only after other medications had been tried and failed. But because they can cause a dramatic improvement in some children with SJIA, some physicians now use biologics as a first-line therapy, avoiding corticosteroids and their side effects completely.

Thomas J.A. Lehman, MD, chief of pediatric rheumatology at Hospital for Special Surgery in New York City, says, "Corticosteroids should no longer be the first-line therapy for severe SJIA. Corticosteroids will remain necessary for children who fail biologic agents, but at least one biologic should be utilized before corticosteroids in (nonemergency) situations."

Daniel J. Lovell, MD, associate director of the division of rheumatology at Cincinnati Children's Hospital, agrees, saying, "Physicians have to be comfortable that a child has SJIA before starting any treatment, but once they are comfortable, many start with a biologic instead of (cortico)steroids.”

Once systemic symptoms of SJIA have disappeared, nonbiologic disease-modifying antirheumatic drugs (DMARDs) may be used alone or in combination with biologics for continued therapy for arthritis. Unlike NSAIDs or corticosteroids, traditional DMARDs may slow joint damage. “They aren’t usually effective in early stages of SJIA, however, and generally don’t improve systemic systems,” says Dr Lovell.

Complex Disease, Personalized Approaches

Systemic juvenile idiopathic arthritis is a complicated disease, and children can have different combinations of systemic symptoms and arthritis with varying levels of severity—all of which must be taken into account when deciding on an approach to SJIA treatment. Philip Kahn, MD, a pediatric rheumatologist and assistant professor of pediatrics at New York University Langone Medical Center, says children with occasional fever and rash and mild arthritis might respond to treatment with only NSAIDs, whereas those with serious systemic symptoms or complications require more aggressive treatment.

"Early, aggressive therapy is appropriate for some patients but not all. I think that tailoring the treatment to the individual is the best approach," Dr Kahn explains.

Meanwhile, the Childhood Arthritis and Rheumatology Research Alliance (CARRA), a network of more than 400 pediatric rheumatologists, is working to determine which treatments best address the needs of children with different SJIA symptoms. CARRA is an Arthritis Foundation research partner.

Shared Decision Making

Having more and alternative SJIA treatment options has improved the outlook for kids with SJIA, but it has also made medication decisions more challenging. Drugs for SJIA differ in how they work, how they are taken, and in their safety and costs. Biologics, as a group, carry a risk of serious infections, for example, and though they have fewer short-term side effects than corticosteroids, their safety over time isn't known. Treatment with an immunosuppressive drug, including biologics, may result in an increased risk of some cancers. They are also administered differently—as a daily or monthly shot or biweekly infusion rather than as a pill and are more expensive than other SJIA drugs.

Faced with choices, parents are likely to have difficulty figuring out which medication would be the safest and most effective for their child. Shared decision making can help make that process easier. In shared decision making, physicians describe all the treatment options—not just the ones they prefer—and families share what is most important to them. Working together, physicians and families then develop a treatment plan. Research has shown that this approach leads to better overall outcomes, says Esi Morgan DeWitt, MD, a pediatric rheumatologist and associate professor at Cincinnati Children's Hospital Medical Center. 

Dr DeWitt and colleagues have identified questions that are important for parents to ask as part of the shared decision-making process. They include

  • How often are they taken?

  • How soon will the medication take effect?

  • What are the potential risks and side effects?

  • What does it cost?

  • How long will it need to be taken?

  • What happens if it doesn’t help?

Dr DeWitt says exploring the answers to these questions can help families and physicians find a medication that best fits each child's needs.

Nondrug Therapies

Although medication is the mainstay of SJIA treatment, a regular exercise program is an important part of therapy, too. Exercise helps to build muscle strength, increase energy, and reduce pain. It can also help kids feel more confident about their physical abilities, provide an opportunity to interact with other children, and help maintain joint function and flexibility.

Most kids can participate fully in physical activities and team sports when their symptoms are well controlled, but they may have to limit certain activities during disease flares. A rehabilitation or physical therapist will likely be part of a child's treatment team and can recommend the best activities.

SJIA Treatment Goals

The goals of SJIA treatment have always been to minimize active inflammation, protect joints, and maintain function. But now the ideal is to achieve remission as quickly as possible.

Yukiko Kimura, MD, chief of pediatric rheumatology at Joseph M. Sanzari Children’s Hospital in Hackensack, NJ, says, "There has been a medication revolution in arthritis treatment since the introduction of biologics. And we have learned that treating with these medications very early and aggressively can dramatically change the course of the disease."

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