Nearly 300,000 children in the United States have some form of arthritis or pediatric rheumatic disease. The most common type is juvenile idiopathic arthritis (JIA), which develops before age 16. Idiopathic means the cause of the disease isn’t entirely clear. There are six JIA subtypes. About 10 percent to 20 percent of children with JIA have a rare and serious subtype called systemic juvenile idiopathic arthritis, or SJIA. “Systemic” means it affects not only the joints but also other parts of the body, including the liver, lungs, and heart.
SJIA, sometimes referred to as Still’s disease, can occur any time during childhood, but it most commonly starts at about 2 years of age. Boys and girls are equally affected. SJIA also differs from other subtypes in that it’s the only one considered an autoinflammatory rather than an autoimmune disease. SJIA is more severe and can be more challenging to diagnose and treat than other types of juvenile idiopathic arthritis. It is a lifelong disease for many patients and can continue into adulthood.
Exactly what causes SJIA isn't clear. The general theory is that there is a genetic predisposition, and the disease onset is triggered by an environmental trigger. But what the trigger or triggers might be remains unclear. In the past few years, researchers have learned a great deal about the inflammatory processes that drive it. READ MORE
The first sign of systemic juvenile arthritis is usually a daily, spiking fever and a fleeting, salmon-colored rash that may appear weeks or months before joint pain does. READ MORE
For about 50 percent of children, the disease goes into permanent remission (no clinical signs and symptoms of active disease) within a few months to a few years. A small number of children experience recurring periods of active disease and remission. But approximately 30 percent of kids have a more severe and persistent disease course. READ MORE
While the disease is active, whether early on or later, inflammation can cause permanent damage to joints. That’s why early, aggressive treatment is so important. The goal of SJIA treatment is to achieve remission as quickly as possible. READ MORE
Although the potential for remission and good outcomes for children with SJIA is more positive than ever before, it is still a severe and complex disease requiring specialty medical care. It’s important for parents to work with a pediatric rheumatologist. There are a limited number of pediatric rheumatologists in the United States, however. When there is no specialist within a reasonable distance, some pediatric rheumatologists practicing out-of-town or out-of-state are willing to coordinate care with a local adult rheumatologist, do remote consultations, or travel to underserved areas. Find a pediatric rheumatologist