What is the initial management for a child or adolescent diagnosed with juvenile rheumatoid arthritis (JRA)?

Initial Management of Juvenile Idiopathic Arthritis

For children with oligoarticular JIA, begin immediately with scheduled NSAIDs and intraarticular glucocorticoid injections (IAGCs) as first-line therapy, escalating to methotrexate if inadequate response occurs within 3 months, and reserve biologic DMARDs for those failing both NSAIDs/IAGCs and at least one conventional DMARD. 1, 2

Diagnostic Approach

• Confirm the diagnosis through clinical assessment of persistent joint swelling, morning stiffness, and functional limitation, recognizing that no single blood test confirms JIA 3, 4
• Classify the JIA subtype (oligoarticular, polyarticular, systemic, or enthesitis-related) as this fundamentally determines the treatment algorithm 1, 2
• Obtain baseline laboratory studies including CBC with differential, ESR, and CRP to assess inflammation and establish baseline values before initiating therapy 2
• Perform imaging with plain radiographs and ultrasound to detect effusion, assess for erosive disease, and evaluate other joint involvement 5
• Identify poor prognostic features including involvement of ankle, wrist, hip, sacroiliac joint, or TMJ; erosive disease; enthesitis; elevated inflammation markers; and symmetric disease, as these warrant more aggressive initial therapy 1, 2

Initial Treatment by JIA Subtype

Oligoarticular JIA (≤4 joints)

• Start scheduled NSAIDs immediately (naproxen is most commonly used) as adjunct therapy while expediting rheumatology referral 1, 2
• Add intraarticular glucocorticoid injections as part of initial therapy, with triamcinolone hexacetonide strongly preferred over triamcinolone acetonide 2, 6
• Avoid oral glucocorticoids as initial therapy; they are conditionally recommended against except for short-term bridging (<3 months) in high disease activity 1, 2
• Escalate to methotrexate if inadequate response to NSAIDs and/or IAGCs after an adequate trial, with methotrexate conditionally recommended as the preferred conventional synthetic DMARD 1, 2, 6
• Consider subcutaneous methotrexate over oral formulation for better bioavailability, particularly at doses >15 mg/m² 6, 7
• Add biologic DMARDs only after inadequate response to or intolerance of NSAIDs/IAGCs and at least one conventional synthetic DMARD 1, 2, 6

Polyarticular JIA (≥5 joints)

• Initiate methotrexate immediately as first-line disease-modifying therapy without delay, rather than NSAID monotherapy 2, 6
• Use subcutaneous methotrexate preferentially over oral administration for improved absorption and efficacy 6, 7
• Continue NSAIDs as adjunct therapy for symptomatic relief 2
• Consider initial biologic therapy only in patients with high-risk joint involvement (hip, wrist, ankle), very high disease activity, or those at high risk of disabling joint damage 6
• Escalate to biologic DMARDs by adding to the original DMARD rather than switching to a second conventional DMARD if moderate/high disease activity persists after 3 months 6
• Switch to non-TNF biologics (tocilizumab or abatacept) over a second TNF inhibitor if the first biologic fails 6

Systemic JIA

• Start NSAIDs as initial monotherapy, conditionally recommended over other options 1, 2, 6
• Strongly avoid conventional synthetic DMARDs as initial monotherapy 2, 6
• Avoid oral glucocorticoids as initial monotherapy 2, 6
• Escalate rapidly to IL-1 or IL-6 inhibitors for inadequate response to or intolerance of NSAIDs and/or glucocorticoids, as these are strongly recommended over conventional synthetic DMARDs 1, 2, 6
• Add biologic or conventional DMARDs for residual arthritis with incomplete response to IL-1/IL-6 inhibitors, strongly preferred over long-term glucocorticoids 6

Enthesitis-Related Arthritis

• Initiate NSAIDs as strongly recommended first-line therapy 2
• Add physical therapy for those with or at risk for functional limitations 2
• Use TNF inhibitors for sacroiliitis, strongly recommended over other options 2
• Prefer TNF inhibitors over methotrexate or sulfasalazine for active enthesitis despite NSAIDs 2

Methotrexate Dosing and Monitoring

• Dose methotrexate at 10-15 mg/m² weekly for JIA, with doses up to 30 mg/m² showing good oral absorption 7
• Allow 3 months for an adequate trial of methotrexate, though changing or adding therapy may be appropriate if no or minimal response after 6-8 weeks 2, 6
• Supplement with folic acid to reduce toxicity, though high doses may theoretically reduce efficacy 7
• Monitor closely for hepatotoxicity, bone marrow suppression, and pulmonary toxicity, particularly in elderly patients or those on concomitant NSAIDs 7
• Discontinue immediately if significant drop in blood counts (WBC <3000/mm³, platelets <100,000/mm³) or signs of hepatotoxicity occur 7

Treatment Monitoring and Targets

• Use validated disease activity measures such as cJADAS-10 to guide treatment decisions and facilitate treat-to-target approaches 2, 6
• Define low disease activity as cJADAS-10 ≤2.5 with ≥1 active joint 2
• Define moderate/high disease activity as cJADAS-10 >2.5, warranting treatment escalation 2
• Reassess at 3-6 weeks after initiating therapy, with clinical improvement typically visible by 3-6 weeks for methotrexate 7
• Escalate therapy if low disease activity persists, with options including IAGCs, DMARD dose optimization, or adding/changing biologics 6

Critical Pitfalls to Avoid

• Never delay DMARD initiation in polyarticular JIA while attempting prolonged NSAID monotherapy, as early treatment prevents permanent joint damage 2, 6
• Never use prolonged oral glucocorticoids as monotherapy; limit to short-term bridging (<3 months) only 1, 2, 6
• Never use preserved methotrexate formulations for intrathecal or high-dose therapy, or in neonates, due to benzyl alcohol toxicity 7
• Never dismiss insidious joint pain as simple overuse without ruling out inflammatory arthritis, as early intervention is crucial 2
• Never combine methotrexate with trimethoprim/sulfamethoxazole without close monitoring due to increased bone marrow suppression risk 7
• Never administer nitrous oxide anesthesia to patients on methotrexate due to potentiated folate-dependent toxicity 7

Adjunctive Therapies

• Refer for physical and/or occupational therapy for children with or at risk for functional limitations 2
• Continue rest and physiotherapy as indicated throughout treatment 7
• Allow continuation of low-dose corticosteroids, aspirin, and NSAIDs during methotrexate therapy, though monitor closely for increased toxicity 7