About Juvenile Idiopathic Arthritis (JIA)
What is juvenile idiopathic arthritis?
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Juvenile idiopathic arthritis (JIA) is a type of autoimmune inflammatory arthritis that can be seen in children under the age of 16 years. Autoimmune diseases generally occur when the body's immune system begins to malfunction and attacks healthy tissue in various parts of the body, causing inflammation and damage. In JIA, joints can become stiff, painful, and swollen. In some severe subtypes of JIA, organs such as the eyes, heart, lungs or skin can be involved.
Who can develop juvenile idiopathic arthritis?
As science has evolved, researchers have come to learn that most diseases in childhood are not the same as their “adult” counterparts. Previously called juvenile rheumatoid arthritis, JIA affects up to three in 1000 children and is one of the most common chronic diseases in childhood1
Overall, JIA affects girls more often than boys1
. JIA can affect children of any age, from infancy to 16 years. While the cause for JIA remains unknown, there are effective treatments that can often lead to remission and prevent permanent joint damage and disability.
The main symptoms of juvenile idiopathic arthritis
The most common complaints of children at the time they develop JIA are joint pain, joint swelling or joint stiffness. Other warning signs that may be present at the onset of disease include2,3
Getting a diagnosis of juvenile idiopathic arthritis
- Change in ability to keep up with normal activities, such as participate in sports or school because of physical joint pain
- Irritability, especially in young children in pain
- Refusal to walk, noticeably limping, or in young toddlers, a return to crawling
There are seven subtypes of JIA, defined by the International League of Associations for Rheumatology (ILAR) international criteria4
- Oligoarticular – the most common form of the disease. About half of children with JIA are diagnosed with the oligoarticular form. Generally, one to four joints are affected. Most common joints include knees, ankles, or wrists. Up to 20% of children diagnosed with the oligoarticular form of JIA will develop uveitis (inflammation in the eyes) which often has no symptoms.
- Extended oligoarticular – approximately 30% of children whose JIA starts in fewer than 4 joints will develop arthritis in more than 4 joints at some point; this is called ‘extended’.
- Polyarticular (rheumatoid factor positive and rheumatoid factor negative) – JIA affecting five or more joints. Any joint can be involved, and usually the arthritis is symmetric (the same on both sides of the body). Children who have a positive rheumatoid factor (RF) have a more persistent and severe type of polyarticular disease.
- Enthesitis-related arthritis – this form of JIA occurs more commonly in school-aged or older children, and affects boys more than girls. Arthritis can affect large joints such as the hip, knees, ankles, as well as the spine and neck. Some of these children have inflammation of ligament and tendon attachments, called enthesitis. A small percentage of these children go on to develop ankylosing spondylitis.
- Psoriatic arthritis – children may have arthritis and psoriasis, or arthritis and a strong family history of psoriasis. This form of arthritis can affect any joint.
- Systemic onset – the most severe form of JIA, affecting about 10% of children diagnosed with juvenile idiopathic arthritis. Children with systemic onset JIA often present with high fever, rash, and inflammation of organs such as the lining of the heart or lungs, in addition to inflammation in the joints.
At the outset, JIA symptoms may be similar to those of other diseases, such as infections, cancer, bone conditions, and other inflammatory illnesses. In addition to a full physical examination and medical history, doctors may order tests such as blood work and x-rays to exclude other possibilities and confirm a tentative diagnosis of JIA. It is important to recognize, however, that there are no blood tests that can confirm the diagnosis of JIA.
Once a diagnosis of JIA is suspected, the child should be referred to a pediatric rheumatology team, a group which includes a pediatric rheumatologist, nurses, pediatric physiotherapist, pediatric occupational therapist, and social worker. These specialists will work together to confirm a diagnosis, design a personalized treatment plan for the child, and work together with the parents/guardians and child to carry out the treatments to maintain joint health.
Treatment for juvenile idiopathic arthritis
While there is no cure for JIA, treatment advances are allowing more children to live normal lives. Medications have been developed that can reduce pain and even slow or stop the inflammation that causes devastating joint damage. As well, physical and occupational therapy can help to allow children to participate in normal activities, and to prevent long-term disability.
There are a number of medications commonly used to treat JIA5,6
. Some manage the symptoms, and others treat the underlying disease process.
- Treatments that help manage symptoms include non-steroidal anti-inflammatory drugs (NSAIDs). Naproxen (Naprosyn®) is the most common NSAID used for treating JIA, but others are used as well.
- Treatments that target the underlying problem include disease-modifying anti-rheumatic drugs (DMARDs). Examples of these include hydroxychloroquine (Plaquenil®), sulfasalazine (Azulfadine®), leflunomide (Arava®) and most commonly methotrexate (Rheumatrex®). DMARDs work to slow or halt the inflammation and disease progression that can cause devastating joint damage and disability. Many children have an excellent response to one, or a combination, of these medications.
- For some children, traditiona1 DMARDs alone will not be enough to control their disease. In these cases, a biologic response modifier may be used. Biologics are the newest, most powerful medications to treat inflammatory arthritis. Examples of these medications are etanercept (Enbrel®) and infliximab (Remicade®). Newer biologic medications are currently being studied in children with JIA. Biologics are often used in combination with traditional DMARDs.
Many parents/guardians of children with JIA note that giving medications is one of the most difficult parts of coping with the disease7
. Education is such a critical part of a treatment plan for JIA. Like many inflammatory conditions, early aggressive treatment is vital. Caretakers who are well informed are more likely to be able to help create and implement a well-rounded, effective treatment plan. It is important to remember that children who are treated quickly and effectively have the very best chance of recovering fully and maintaining joint health. The pediatric rheumatology team, especially the nurse, is usually a great support for parents as they learn to support their child.
Physiotherapy and occupational therapy8
Physiotherapy and occupational therapy are very important treatment plan components for every child with JIA. The physiotherapist and occupational therapist will work on regaining range of motion in stiff joints, providing techniques for pain relief, splinting when required, and once the disease is controlled, an exercise plan. Further, physiotherapist and occupational therapist can help to adapt everyday activities to be more accessible for children living with JIA.
Exercise and play are key component of a well-rounded treatment plan for JIA and should be skillfully balanced with adequate rest. Exercise can help maintain joint health — keeping them mobile— and rest can help with recovery and adaptation. Children with JIA are encouraged to participate in usual activities, such as physical education and team sports at school, to the best of their abilities. If symptoms are well controlled, very little is off limits. However, movements that involve certain joints with arthritis can make them less suitable. For example, basketball may be uncomfortable with active arthritis in the knees or ankles. Finding the right fit might take some time and that is okay. Start with low impact activities that challenge the cardiovascular system including swimming and cycling. Remember children with JIA are outstanding athletes, they simply require patience and encouragement like anyone else.
The importance of a nutrient rich diet is well recognized in children with JIA to support physical and mental growth. It is thought that whole foods help the body recover and reduce inflammation in JIA patients. Thus, a balanced meal includes a good balance of complex carbohydrates, fruits, vegetables, and healthy fats. Inflammatory foods that contain artificial ingredients and excess amounts of sugar should be minimized to avoid the risk of additional health problems.
Thank you to Dr. Mercedes Chan, pediatric rheumatologist, BC Children’s Hospital, for her medical review of the content on this page.
- The outcome for children with JIA has improved over the past years, especially if the disease is recognized and treated effectively in the early stages of the disease process.
- Programs are available to help children living with JIA transition to adult arthritis models of care. To learn more, watch this episode of Arthritis At Home with Dr. Elizabeth Hazel.
- An important part of managing JIA is educating others about the disease and helping them understand how JIA impacts the child, their families and friends, and their school life.
|5||Onel et al. (2022). 2021 American College of Rheumatology guideline for the treatment of juvenile idiopathic arthritis: therapeutic approaches for oligoarthritis, temporomandibular joint arthritis, and systemic juvenile idiopathic arthritis. https://doi.org/10.1002/art.42037|
|6||Onel, et al. (2022). 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Therapies, Medication Monitoring, Immunizations, and Imaging. https://doi.org/10.1002/art.42036|
|7||Guzman et al. (2014). What matters most for patients, parents, and clinicians in the course of juvenile idiopathic arthritis? A qualitative study. https://doi.org/10.3899/jrheum.131536|