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There are various subtypes of juvenile idiopathic arthritis, one of which is systemic juvenile idiopathic arthritis. This article outlines the causes, symptoms, diagnosis and treatment of systemic juvenile idiopathic arthritis.

Arthritis is a common condition that affects more than 300,000 children in the United States alone. Arthritis in children is known as juvenile idiopathic arthritis, which is made up of six individual subtypes. One of these, systemic juvenile idiopathic arthritis, affects between 10 and 15 percent of all children with juvenile idiopathic arthritis.

While it is known that systemic juvenile idiopathic arthritis can occur at any point during childhood, it typically develops before a child turns five, with the most common age of diagnosis being two.

Causes of systemic juvenile idiopathic arthritis

Systemic juvenile idiopathic arthritis is an autoimmune disease, which means that it develops as a result of an impaired immune system. Generally, the immune system works to keep us healthy by attacking foreign objects such as bacteria or viruses. In the case of autoimmune disease, however, the immune system confuses healthy tissue for a foreign substance and starts to attack it. Autoimmune diseases are characterized by high levels of inflammation, and in the case of arthritis, this inflammation tends to occur around the joints and causes them to become red, painful and swollen. However, in the case of systemic juvenile idiopathic arthritis, the inflammation extends beyond the joints and affects different organs in the body such as the heart, liver, and spleen.

While we know that systemic juvenile idiopathic arthritis is an autoimmune disease, it is not clear what triggers the development of this disease, hence it's called idiopathic. Possible triggers can include a combination of genetic and environmental factors.

Symptoms: Can you recognize juvenile idiopathic arthritis?

The symptoms associated with the development of systemic juvenile idiopathic arthritis are:

  • Painful, swollen and inflamed joints
  • Fevers, which can last for a long time and recur. A high fever that keeps recurring is one of the first signs that a child has systemic juvenile idiopathic arthritis. Fever may strike once or twice a day, and the pattern is that the fever reaches 103 degrees or more in the evenings, and drops after. Symptoms of arthritis may occur alongside fever or later.
  • A pink-colored, scaly skin rash. This usually develops in the trunk, arms, or legs. It may also be itchy.
  • Being tired or fatigued.
  • Widespread muscle aches and pain.
  • Inflammation of internal organs such as the heart, spleen and liver.
  • Enlargement of lymph nodes.
  • Anemia (a low red blood cell count).

Diagnosis: How is juvenile idiopathic arthritis diagnosed?

If your family doctor suspects that your child has juvenile idiopathic arthritis, they will refer them to a specialist known as a rheumatologist. The doctor will look for signs of joint pain or swelling, skin rashes, fever, enlarged lymph nodes, and organ inflammation. The rheumatologist will make a diagnosis based on description of the symptoms, medical history, physical examination and various types of tests.

While there is no single test that can help diagnose systemic juvenile idiopathic arthritis, a range of tests that can help determine the cause of these symptoms. These tests include:

  • Blood tests, to look for red and white blood cell counts and signs of inflammation
  • Urine tests
  • Joint fluid tests
  • Imaging tests such as X-rays and MRIs

Treatment: How is juvenile idiopathic arthritis managed?

Systemic juvenile idiopathic arthritis can be quite a severe condition, and children often have to be hospitalized to treat some of the extreme symptoms such as high fever. While there is no cure for arthritis, there are treatments that can be administered to help treat the symptoms of the disease. These include:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs), which include ibuprofen (Advil) and naproxen (Aleve). NSAIDs help ease symptoms of arthritis by reducing pain, fever, and inflammation.
  • Corticosteroids, such as prednisone, help relieve inflammation by modulating the autoimmune response. These can be administered intravenously (into the vein), orally (through a tablet) or right into the inflamed joint through an injection.
  • Biologic therapy can help treat the underlying condition. For example, a common kind of biologic, known as TNF inhibitors, inhibit an inflammatory molecule called TNF-alpha that helps relieve inflammation and swelling.
While these different medicines can be good symptomatic treatment, it is important to keep in mind that they can carry significant side effects and that if your child is experiencing any of these side effects, it is important to inform your doctor so that they can lower the dose or discontinue the drug.

Other non-medical treatments for systemic juvenile idiopathic arthritis include:

  • Rest, which is very important for children with this disease, especially when they are actively experiencing symptoms such as fever.
  • Exercise, which can help keep their joints and muscles working. This can be done in conjunction with a physical therapist that can work with your child to come up with an individualized exercise program.

This disease — its severity, symptoms, and course — can vary from individual to individual. While some patients will experience symptoms for a short amount of time before finding complete relief, others can continue to experience symptoms into adulthood. While researchers are unsure why some people are less affected than others, studies point a role for genetics in this area.

  • Ravelli, Angelo, and Alberto Martini. "Juvenile idiopathic arthritis." The Lancet 369.9563 (2007): 767-778.
  • Mellins, Elizabeth D., Claudia Macaubas, and Alexei A. Grom. "Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions." Nature Reviews Rheumatology 7.7 (2011): 416.
  • Schneider, Rayfel, and Ronald M. Laxer. "6 Systemic onset juvenile rheumatoid arthritis." Bailliere's clinical rheumatology12.2 (1998): 245-271. Photo courtesy of SteadyHealth.com

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