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Signs and Symptoms of Systemic Juvenile Idiopathic Arthritis

Signs and Symptoms of Systemic Juvenile Idiopathic Arthritis
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Recognizing the symptoms of systemic juvenile idiopathic arthritis (SJIA) can help kids get diagnosed and treated sooner.

The symptoms of SJIA were first identified by British pediatrician Sir George Still more than a century ago. He noticed that some patients—most of them younger than 3 years old—had an unusual group of symptoms that included arthritis and high fevers. Today, these are still the defining signs and symptoms of SJIA.

Recurrent Fever and Rash

Although SJIA can occur any time during childhood, it usually starts before age five—most commonly at about 2 years of age. In almost every case, a high, recurring fever, often with a rash, is one of the first signs of SJIA. The fever usually follows a pattern in which a child’s temperature reaches 103 degrees or higher, generally in the evening, and then drops within a few hours.

Although a daily, spiking fever, typically in the evening, is one of the criteria for diagnosing SJIA, studies have shown that the pattern can vary. Sometimes the fever occurs in the morning or twice a day; occasionally, it may continue throughout the day. Over time, though, almost all children with untreated SJIA develop the typical pattern of daily fever that returns to normal.

Fevers are often accompanied by a flat, pale, pink rash that frequently appears on the child’s trunk, arms, or legs, although it can move from one part of the body to other parts.The rash may be itchy, but usually isn’t. It tends to last a few minutes to a few hours and is associated with fever spikes. Tracking the symptoms of SJIA and sharing them with doctors can help them make an accurate SJIA diagnosis.

Other Systemic Symptoms of Systemic Juvenile Idiopathic Arthritis

In addition to fever and rash, SJIA is associated with systemic signs such as swollen lymph nodes, enlarged liver or spleen, and sometimes inflammation of the lining of the heart (pericarditis) or lungs (pleuritis). Pericarditis may be an early sign in 10 percent of children. For some children, systemic symptoms disappear, leaving chronic, progressive arthritis as the primary condition.

How Systemic Juvenile Idiopathic Arthritis Affects the Joints

Arthritis is the second most common early sign of SJIA. The symptoms of joint swelling, pain, stiffness, and warmth that occur are worse in the morning and after a nap or prolonged stillness. However, unlike other forms of childhood arthritis, joint problems may develop weeks or even months after systemic symptoms, making diagnosis challenging. Children, especially very young ones, often don’t complain of joint pain with SJIA, but parents usually notice the onset of arthritis when a child starts to limp, seems stiff in the morning, or suddenly becomes less active.

Sometimes a single joint is affected, but more often several joints are involved, commonly the knees, wrists, and ankles. Children with SJIA can also develop arthritis in the spine (in the neck area), jaw, and hip joint, where it can be very destructive, leading to the need for early hip replacement surgery. In general, the more joints affected by arthritis, the more severe the disease. About 30 percent of kids develop chronic polyarthritis that may continue into adulthood.

In addition to stiffness and pain, arthritis can cause growth issues. Depending on the disease severity and joints involved, one arm or leg may be longer than the other or overall growth may be slowed. This can result from the disease as well as corticosteroid drugs, which are sometimes used to treat SJIA.

Early Systemic Juvenile Idiopathic Arthritis Diagnosis and Treatment Are Key

Not too long ago, SJIA was almost always crippling. Today, thanks to a better understanding of the disease and additional treatment options, that’s no longer the case, explains Carol A. Wallace, MD, a professor of pediatrics at the University of Washington School of Medicine and Seattle Children’s Hospital.

Still, for kids with SJIA to have the best outcomes, early diagnosis and treatment are essential. The severity of SJIA in the initial stages varies. Some children are very sick when their fever spikes in the evening, but feel fine at other times. Other kids have severe, persistent, systemic illness and complications that may require hospitalization and sometimes intensive care at the onset of the disease or a later stage. It’s important to develop an effective treatment plan with a pediatric rheumatologist or an adult rheumatologist who has experience diagnosing and treating juvenile arthritis. The Arthritis Foundation is working to increase the number of pediatric rheumatologists and can help you find the specialist closest to you.

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