What are the latest European League Against Rheumatism (EULAR) guidelines for managing Juvenile Idiopathic Arthritis (JIA)?

Latest EULAR Guidelines for Managing Juvenile Idiopathic Arthritis (JIA)

The most recent comprehensive guidelines for managing Juvenile Idiopathic Arthritis come from the 2019 American College of Rheumatology/Arthritis Foundation recommendations, which are widely used internationally in the absence of newer EULAR-specific guidelines for non-systemic JIA. 1

Classification and Disease Activity Assessment

• JIA is defined as arthritis of unknown etiology beginning before age 16 years and persisting for at least 6 weeks, with other known conditions excluded 1
• For treatment purposes, patients are grouped by clinical phenotypes rather than strict ILAR categories: polyarthritis (≥5 joints), sacroiliitis, and enthesitis 1
• Disease activity is assessed using the clinical Juvenile Disease Activity Score based on 10 joints (cJADAS-10):
  • Low disease activity: cJADAS-10 ≤2.5 with ≥1 active joint 1
  • Moderate/high disease activity: cJADAS-10 >2.5 1

Treatment Recommendations for Polyarticular JIA

Initial Therapy

• Initial NSAID monotherapy is no longer recommended for polyarthritis due to established benefits of early DMARD initiation 1
• Methotrexate is conditionally recommended as first-line DMARD therapy 1
  • Subcutaneous administration is preferred over oral route 1
  • An optimal trial is considered 3 months, but therapy can be changed after 6-8 weeks if minimal/no response 1

Adjunctive Therapies

• NSAIDs are conditionally recommended as adjunct therapy only 1
• Intra-articular glucocorticoid injections are conditionally recommended as adjunct therapy 1
  • Triamcinolone hexacetonide is strongly preferred over triamcinolone acetonide 1
• Short-term oral glucocorticoid bridging (<3 months) is conditionally recommended during initiation or escalation of therapy in moderate/high disease activity 1
• Physical and occupational therapy are recommended for patients with or at risk for functional limitations 1

Subsequent Therapy

• For inadequate response to methotrexate, adding a biologic DMARD is recommended 1
• Combination therapy with methotrexate plus a biologic is conditionally recommended over biologic monotherapy for most biologics 1
• Combination therapy with methotrexate is strongly recommended when using infliximab 1
• Biologic options include:
  • TNF inhibitors (etanercept, adalimumab, golimumab)
  • IL-6 receptor inhibitor (tocilizumab)
  • T-cell co-stimulation modulator (abatacept) 1

Treatment Recommendations for Sacroiliitis

• TNF inhibitors are conditionally recommended as initial therapy over NSAIDs or methotrexate 1
• For patients with active sacroiliitis despite NSAIDs, TNF inhibitors are strongly recommended over methotrexate or sulfasalazine 1
• Physical therapy is conditionally recommended for patients with or at risk for functional limitations 1

Treatment Recommendations for Enthesitis

• NSAIDs are conditionally recommended as initial therapy 1
• For patients with active enthesitis despite NSAIDs, TNF inhibitors are conditionally recommended over methotrexate or sulfasalazine 1
• Bridging therapy with limited course of oral glucocorticoids (<3 months) is conditionally recommended during initiation or escalation of therapy 1
• Physical therapy is conditionally recommended for patients with or at risk for functional limitations 1

Treatment Goals and Monitoring

• The primary goal is achieving low disease activity or remission to prevent joint damage and disability 1
• Therapeutic escalation is recommended to achieve low disease activity 1
• Regular monitoring of disease activity, functional status, and treatment-related adverse effects is essential 1

Special Considerations for Systemic JIA

• While not the focus of the 2019 guidelines, recent evidence supports IL-1 inhibitors and IL-6 receptor inhibitors as having the highest level of evidence for efficacy and safety in systemic JIA 1
• For macrophage activation syndrome (MAS), a potentially life-threatening complication, high-dose glucocorticoids combined with IL-1 or IFN-γ inhibition appear to be the best available strategy 1

Important Caveats

• Most recommendations (31 of 39) in the 2019 guidelines are conditional, based on low or very low quality evidence 1
• Treatment decisions should consider patient values, preferences, and comorbidities 1
• The guidelines should not be used to limit or deny access to therapies 1
• Early aggressive treatment with biologics may be effective but could lead to overtreatment in patients who would respond to conventional DMARDs 2
• There remains a lack of evidence for optimal treatment of some JIA subtypes, particularly systemic and enthesitis-related arthritis 3