Systemic Juvenile Idiopathic Arthritis (SJIA)

What is arthritis?

Arthritis means “joint swelling” or “joint inflammation”. Arthritis is a group of diseases that cause pain, swelling, stiffness and loss of motion in the joints.

Juvenile idiopathic arthritis (JIA)

Juvenile idiopathic arthritis (JIA) affects children who are less than 16 years old. It makes the joints inflamed and stiff for more than six weeks. “Idiopathic” means that we do not know the cause of the disease.

Systemic juvenile idiopathic arthritis (SJIA)

There are many types of JIA. Systemic juvenile idiopathic arthritis (SJIA) is one type. Between 10% and 15% of all children with JIA have SJIA.

SJIA is an autoimmune disorder

SJIA is an autoimmune disorder, like all types of arthritis. This means that the body mistakes some of its own cells and tissues as foreign. The immune system normally helps to fight off harmful, foreign substances such as bacteria or viruses. When a child has SJIA, the immune system begins to attack healthy cells and tissues. This results in inflammation. Inflammation is when a part of the body is red, swollen, painful, or hot to the touch.

Symptoms of SJIA include fevers, rash, and joint pain

Children who have SJIA get recurrent fevers, which means the fevers go away but come back at a later time. Fevers usually occur once or twice per day. When a fever is at its worst, a child may have a pink-coloured skin rash and may feel tired and achy or have widespread aches and pains.

SJIA can also cause inflammation or swelling of the internal organs such as the heart, liver, spleen, and lymph nodes. It is common for children with SJIA not to have enough red blood cells (anemia). They may also have too many white blood cells. Stiffness and pain in the joints (arthritis) may occur at the same time or later on.

SJIA is diagnosed with blood tests and other investigations

Doctors may suspect SJIA when they see children who have ongoing joint pain or swelling, unexplained skin rashes and fever, swelling of lymph nodes, or inflammation of internal organs. However, these problems can be caused by other diseases. Doctors will usually order blood tests, X-rays, and sometimes additional tests to help diagnose the disease.

SJIA may need a variety of medicines

Children with systemic arthritis are often ill. Some may have to stay at the hospital while the disease and its symptoms are treated. Treatments control both the arthritis and other parts of the body affected by the disease.

The drugs most commonly used to treat SJIA are:

There may be other treatments as well. Researchers are always learning more about SJIA and how to treat it.

Most drugs have some side effects. These are effects that are not part of the treatment. If your child is given any drug, the doctors or nurses will let you know about the drug’s side effects and what that might mean for your child.

Living with SJIA

SJIA may affect your child’s activities

SJIA may affect your child’s usual activities. The pain and stiffness of joints may make normal tasks hard to do. For example, SJIA may make it harder for your child to do things at school. It may also affect your child's social life. Part of treatment for SJIA will be to try to lessen the effect of SJIA on your child's usual activities.

SJIA may affect your child at school

Some children with more severe arthritis may not be able to take part in school activities. They may even have trouble writing. It may also be harder for them to get to school. They may have to miss school days because they are in the hospital or going to doctors' appointments.

Let the school know about your child's illness. They may be able to make your child's school life easier. Your child’s doctor can also contact the school to help make things easier. For example, the doctor may help to arrange access to an elevator, transportation, or an extra set of school books to use at home.

Your child's SJIA can affect the whole family

When a child has a disease, it can affect the whole family. The beginning of SJIA is often sudden and can be scary to both the child and the family. Sometimes the disease seems to get better, and at other times it suddenly seems to get worse. This can be hard on the whole family.

A child with SJIA, the parents, or the child’s brothers or sisters may sometimes feel guilty about what has happened. Other times, a brother or a sister may resent the extra attention a child with SJIA gets from his or her parents. If these things happen in your family, you can speak to a counsellor or a doctor. They may be able to help you through these hard times and may give you ideas to make things a little easier on everyone.

Try to keep your child with SJIA doing the same things that he or she did before the disease, whenever possible. If you are unsure of what your child can do, ask one of your health care team members. Daily routines are important to children. Routines can include chores, school, visits with friends, and play time. If routines can change as little as possible, this will help your child feel better about how the disease has affected him or her. Social workers can give you advice on how to keep routines from changing too much. They can help you, your child, and your family set realistic goals.

Follow-up care at the rheumatology clinic

Your child will be referred to a rheumatology clinic for follow-up care. Usually, a rheumatology clinic will have a team made up of doctors and nurses who have a lot of experience treating children with SJIA.

Other members of the rheumatology team may include:

Preparing for visits to the rheumatology clinic

Your child may need a blood test during a clinic visit. This will help doctors monitor the disease and check for possible side effects of the medicine your child may be taking. If your child needs to prepare for clinic visits in any other way, a member of the rheumatology team will tell you before the visit.

After you have met the rheumatology team, you will know more about how to care for your child and how to plan for future clinic visits.