For years, physicians have treated systemic juvenile idiopathic arthritis – a serious, complex, but rare disease – based on limited scientific data and their own experience. But recently, as the number of effective medicines to control systemic juvenile idiopathic arthritis, or sJIA, have increased, an organization of leading pediatric rheumatologists has decided that sJIA treatment should be standardized. By establishing treatment plans, the physicians hope patient care will improve and future research will be made easier.

“The Consensus Treatment Plans for New-Onset Systemic Juvenile Idiopathic Arthritis” – published online recently in the journal Arthritis Care & Research – was assembled by a core workgroup from the Childhood Arthritis and Rheumatology Research Alliance, or CARRA, a clinical research network whose more than 300 members are affiliated with 92 centers in North America.

“There is wide variation in therapeutic approaches to [sJIA],” the project authors write. “Understanding the comparative effectiveness of the diverse therapeutic options available for treatment … can result in better health outcomes” for patients.

Systemic JIA is distinguished from other forms of JIA by serious inflammation in the bloodstream that can affect internal organs and cause high fevers and rashes. Of the approximately 300,000 children with juvenile arthritis in this country, an estimated 10 percent have sJIA. 

The biologic tocilizumab, or Actemra, an IL-6 inhibitor, was the first treatment approved by the U.S. Food and Drug Administration, or FDA, for sJIA in April 2011. Anakinra, or Kineret, an IL-1 inhibitor, is FDA-approved to treat adult rheumatoid arthritis, but is sometimes used “off-label” for sJIA. Other IL-1 inhibitors, such as canakinumab, or Ilaris, and rilonacept, or Arcalyst, are currently in clinical trials to treat sJIA. Biologic drugs already approved for JIA may not be as effective in the treatment of sJIA. 

Although the American College of Rheumatology last April issued recommendations for treating all forms of JIA – before Actemra was approved for sJIA – the CARRA plans aim to further refine treatment approaches for sJIA. The authors hope the four plans presented in the article will help standardize care for children newly diagnosed with sJIA through the first nine months of treatment. But the impact of determining the best, science-backed treatments could last a lifetime.

“There has been a medication revolution in arthritis treatment since the introduction of biologics,” says co-author Yukiko Kimura, MD, chief of pediatric rheumatology at Joseph M. Sanzari Children’s Hospital, Hackensack University Medical Center, in New Jersey, who helped develop the consensus treatment plans. “We have learned that starting to treat with these medications very early may dramatically change the course of a disease that otherwise can be so terribly crippling and disabling and, in the case of systemic JIA, life-threatening.”

They also hope the treatment plans serve as the basis for comparative studies of sJIA therapies. “For the first time, we are trying to persuade pediatric rheumatologists to reduce variations in treatment so we can obtain accurate data on the outcomes and long-term safety of sJIA therapies,” says Carol A. Wallace, MD, professor of pediatrics at the University of Washington School of Medicine and Seattle Children's Hospital and one of the co-authors of the article.

The four plans cover the use – in combination or alone – of four therapeutic agents: corticosteroids, the disease-modifying drug methotrexate, and the biologics anakinra and tocilizumab.