Juvenile idiopathic arthritis is a form of autoimmune arthritis in children. As pharmacists, we can offer support and information for parents, addressing their concerns and helping their children receive appropriate treatment. The following is a brief overview of the condition, treatment options, and counselling tips.
What is juvenile idiopathic arthritis? What are current studies telling us about the disease?
Juvenile idiopathic arthritis (JIA), previously known as juvenile rheumatoid arthritis, generally occurs in children 16 years of age or younger, and includes any form of arthritis of unknown cause with a minimum duration of 6 weeks. Approximately 10,000 Canadian children and teenagers are affected by JIA. Although some children may outgrow this form of arthritis, not all will. There are various subtypes of JIA, including oligoarticular (50%-60% of children with JIA), polyarticular, systemic, enthesitis-related (arthritis also including inflammation where a ligament, tendon or joint capsule is attached to bone), and psoriatic arthritis. In some children the disease is limited to only joints, but in others it may also involve the eyes or skin, affect growth, or less often cause inflammation of internal organs (e.g. heart, liver, spleen, lymph nodes). The condition occurs in flares and is mainly characterized by pain, stiffness, and swelling in the joints, especially after long periods of inactivity (for example, first thing in the morning). In some cases, these symptoms may be accompanied by a fever or rash.
The specific cause of JIA is unknown. Infections, stress, trauma, genetics, and environment are possible factors that may contribute to the development of JIA. There is also growing evidence that the gut microbiome may be involved. One case-controlled study found an association between the use of antibiotics in childhood and an increased risk of developing JIA; it was hypothesized that changes in intestinal microflora may be related to the onset of this autoimmune disorder.¹ More research is needed to confirm these findings.
How is JIA treated? How does it differ from adult rheumatoid arthritis?
As there is no known cure for JIA, the main goals of therapy are to control pain and inflammation, improve joint function, and prevent long-term joint damage. Ideally, treatment should induce remission while avoiding toxicity from medications. Treatment strategies depend on JIA subtype and severity of the disease, but usually involve a combination of medication and physical therapy. The diagnosis of JIA subtype will help determine treatment options, however it is also important to note that the type of JIA may change over time.
If left untreated, inflammation from JIA can permanently damage a joint. Therefore, aggressive early treatment is key to gaining control of the disease. Many of the same drugs for treating adult rheumatoid arthritis are also used in JIA, including NSAIDs, corticosteroids, DMARDs, and biologic agents. However, where methotrexate is generally used first line in adult rheumatoid arthritis, NSAIDs are typically chosen as initial therapy in children and teenagers. If NSAIDs do not control the JIA, a corticosteroid injection may be tried if only a few joints are affected. However, if several joints are affected, oral corticosteroids or DMARDs may be the next step. If the JIA is still not controlled after these options, biologic therapy may be initiated.
Oral or IV corticosteroids have numerous side effects and thus are not ideal in young JIA patients, but they do improve symptoms quickly (e.g., within 48 hrs). If needed, the smallest dose and duration should be used. Systemic corticosteroids are usually reserved for children with severe JIA symptoms while bridging to another medication (like a DMARD), for systemic subtype JIA, or rarely in cases where nothing else has worked. In comparison, intra-articular corticosteroid injections have a lower side effect profile and are preferred when only a few joints are involved, as these provide quick relief of inflammatory symptoms, as well as improve joint function.
DMARDs help control inflammation and also prevent permanent joint damage. They will often be used in combination with other medication as they take anywhere from weeks to 6 months to take effect. Careful and frequent monitoring is necessary to prevent serious side effects like liver damage and low blood cell counts.
Biologic agents are very effective when treating JIA, often with quick improvement in the first couple of weeks. Biologics may help patients resistant to conventional therapy, and are considered safe and effective for JIA patients. Their availability has vastly improved JIA treatment, with decreases in joint damage and increases in disease remission. However, as long-term safety data in children and teenagers is still being studied, it is also key to monitor patients closely, as biologics also have potentially serious side effects.
Additional counselling tips:
VACCINES: Corticosteroids and many of the DMARDs may interact with vaccines, potentially impacting the child’s routine vaccination schedule. For optimal immune protection, administration of inactivated vaccines should be given at least 2 weeks before starting immunosuppressive therapy if possible. Since live vaccines are specifically contraindicated during immunosuppressive treatment, these should be administered either four weeks before or anywhere from 4 weeks to 12 months (or longer) after immunosuppressive therapy. As patients on immunosuppressive therapy have a higher risk of complications from pneumonia and influenza, they should also receive the conjugate pneumococcal vaccine (all ages) and polysaccharide pneumococcal vaccine (if over 2 years old), as well as a yearly inactivated influenza vaccine.
DMARDs or BIOLOGICS: Ensure parents know which health professional to contact if the child gets sick while on these medications. If the child develops any sign of infection such as a fever or chills, cough, sore throat, diarrhea, rash, or is exposed to someone with chicken pox, the doctor should be contacted right away. If on a biologic, it is usually recommended to hold further doses until the child is assessed by a doctor. Teenagers should be cautioned to avoid alcohol due to the potential for drug interactions, and encouraged to use birth control as many of these medications may not be safe in pregnancy.
This post was initially published and titled Juvenile Rheumatoid Arthritis in 2015. Written by:
Fauzia Lalani, BSc Phm, RPh
The title was updated to Juvenile Idiopathic Arthritis and the writing and references were revised and updated in 2021 by:
Chelsea Geen, BScH, MES, PharmD, RPh