A small Dutch study shows that most children with systemic juvenile idiopathic arthritis (sJIA) can achieve inactive disease – and even long-term remission – without the help of corticosteroids, by being treated with the biologic drug anakinra (Kineret) shortly after diagnosis.The observational study, which appears online in Arthritis & Rheumatology, is the first to use anakinra as the initial treatment for sJIA.

Systemic JIA, which affects about 10 percent of children with JIA, is the most severe subtype of the disease. In addition to joint pain and stiffness, it is marked by skin rashes, daily spiking fevers and body-wide inflammation that can damage internal organs.

Corticosteroids have long been a mainstay of sJIA treatment because they reduce inflammation. But they also can cause irreversible side effects in young children, including osteoporosis, diabetes and slow growth. Current recommendations from the American College of Rheumatology (ACR) recommend the use of corticosteroids initially for some patients with sJIA, depending on initial symptoms.

Biologic drugs, once viewed as drugs of last resort, are now considered among the most promising treatments for sJIA. Although one kind of biologic therapy – TNF inhibitors (such as etanercept (Enbrel) and adalimumab (Humira)) – work well for other types of juvenile arthritis, they aren’t particularly effective for sJIA. But biologics that target other pro-inflammatory proteins – such as anakinra, which blocks the pro-inflammatory proteins interleukin-1 (IL-1) and interleukin-18 (IL-18) – have shown more promise. Like the other two IL-1 blockers – canakinumab (Ilaris) and rilonacept (Arcalyst) – anakinra appears to work best when started early in the disease process because it prevents permanent damage (and may change the course of the disease).

Lead study author Sebastiaan Vastert, MD, PhD, a pediatric rheumatologist at the University Medical Center Utrecht, in the Netherlands, is among a growing number of clinicians and researchers who believe biologics, not corticosteroids, should be a first-line therapy for children with sJIA.

“We got the idea to start anakinra in steroid-naive patients because of a good response [to it] in our patients [in whom steroids didn’t work],” Dr. Vastert explains. “Interestingly, we also found in experiments that anakinra seemed to be more effective than [the corticosteroid] prednisolone in lowering IL-18 levels. Both these observations brought us to the idea of starting very early in the disease course, hoping that at some point the biology of the disease could be altered.”

To test this theory, Dr. Vastert and colleagues treated 20 newly diagnosed sJIA patients with daily injections of anakinra as a first-line therapy between 2008 and 2012.

Each study participant was followed for a minimum of one year and some for up to three years. Response to treatment was assessed using an adaptation of ACR criteria, which measure improvements in joint pain and swelling, inflammation markers in the blood, and overall well-being.

For the study, researchers defined “inactive disease” as the absence of fever, fatigue and joint pain/stiffness, and low levels of blood markers of inflammation. Patients were considered to have achieved “clinical remission on medication” when they had inactive disease for six months while still taking anakinra. They were considered to have achieved “clinical remission off medication” when they had inactive disease for 12 months without the use of drugs.