• Responses to treatments for juvenile idiopathic arthritis vary tremendously.
  • Some patients may respond to nonsteroidal anti-inflammatory drugs within 1–2 weeks.
  • Others take 4–6 weeks to improve, and some may not respond at all.
  • Steroids usually start to relieve symptoms within a few days.
  • Methotrexate usually takes 4–8 weeks until a benefit is seen.
  • Anti–tumor necrosis factor therapy can start decreasing symptoms in as little as 1–2 weeks, or it may take up to 3 months.
  • Other second-line agents can take up to 16 weeks until the maximum benefit is seen.
• The waxing and waning nature of juvenile idiopathic arthritis itself adds to the variability of patient responses to treatments.

Diet

• Maintain adequate calcium and vitamin D intake to minimize osteoporosis.
• Patients on methotrexate should take folate supplements daily, except on the days that the methotrexate is given.

Physical Therapy

• Physical and occupational therapy are important in the management of juvenile idiopathic arthritis.
• The goal is to maintain range of motion, muscle strength, and function.

First Line
NSAIDs:

• First-line therapy for juvenile idiopathic arthritis
• If there is no response to the initial NSAID after 4–6 weeks of an adequate dose, a different one should be tried. Patients will often respond differently to the various nonsteroidal drugs.
• If patients experience GI upset or excessive bruising, COX-2 inhibitors may be used.

Second Line
If NSAIDs are ineffective in controlling the disease, a second-line agent should be added, such as methotrexate or sulfasalazine.

• Methotrexate: If the arthritis does not respond to NSAIDs, methotrexate is often started. Laboratory values must be monitored closely in these patients, looking for bone marrow suppression or elevation of transaminase levels.
• Sulfasalazine is most often used in ERA.

OtherThird Line

• Biologic agents are often added when patients do not respond adequately to methotrexate or cannot tolerate its side effects.
• Anti-tumor necrosis factor therapy is frequently used: Etanercept is a receptor for tumor necrosis factor that is given SC once or twice a week. Inflixamab is a chimeric antibody to tumor necrosis factor that is given IV every 4 to 8 weeks. Adalimumab is a fully humanized antibody to tumor necrosis factor given SC every other week.
• Anakinra is a recombinant IL-1 receptor antagonist. It is given as a daily SC injection.
• Abatacept is a co-stimulation blocker. It blocks the interaction of CD28 on T cells with CD80 and CD86 receptors on antigen presenting cells. It is given IV every 4 weeks. It is currently approved for use in adult RA but is still being studied in children.
• Rituximab is an antibody to CD20, which is present on all B cells. It is approved for use in adult RA but not in JIA.
• Anti- IL-6 therapy is currently being studied in children with systemic onset juvenile idiopathic arthritis.
• Medications such as cyclophosphamide or thalidomide are sometimes necessary to control severe systemic-onset juvenile idiopathic arthritis.

OtherGlucocorticoids

• In systemic juvenile idiopathic arthritis with high fevers, systemic glucocorticoids are often necessary, either as oral (daily or every other day) doses or as IV pulses, every 2 to 8 weeks. Systemic steroids are also used for patients with polyarticular juvenile idiopathic arthritis whose arthritis is unresponsive to other medications. Because of the many side effects, patients should be weaned off steroids as soon as possible.
• Intra-articular steroids are often used in patients with only one or a few active joints.