2019 American College of Rheumatology/Arthritis Foundation Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Non-Systemic Polyarthritis, Sacroiliitis, and Enthesitis

Abstract

Objective: To develop treatment recommendations for children with juvenile idiopathic arthritis manifesting as non-systemic polyarthritis, sacroiliitis, or enthesitis.

Methods: The Patient/Population, Intervention, Comparison, and Outcomes (PICO) questions were developed and refined by members of the guideline development teams. A systematic review was conducted to compile evidence for the benefits and harms associated with treatments for these conditions. GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology was used to rate the quality of evidence. A group consensus process was conducted among the Voting Panel to generate the final recommendations and grade their strength. A Parent and Patient Panel used a similar consensus approach to provide patient/caregiver preferences for key questions.

Results: Thirty-nine recommendations were developed (8 strong and 31 conditional). The quality of supporting evidence was very low or low for 90% of the recommendations. Recommendations are provided for the use of nonsteroidal antiinflammatory drugs, disease-modifying antirheumatic drugs, biologics, and intraarticular and oral glucocorticoids. Recommendations for the use of physical and occupational therapy are also provided. Specific recommendations for polyarthritis address general medication use, initial and subsequent treatment, and adjunctive therapies. Good disease control, with therapeutic escalation to achieve low disease activity, was recommended. The sacroiliitis and enthesitis recommendations primarily address initial therapy and adjunctive therapies.

Conclusion: This guideline provides direction for clinicians, caregivers, and patients making treatment decisions. Clinicians, caregivers, and patients should use a shared decision-making process that accounts for patients' values, preferences, and comorbidities. These recommendations should not be used to limit or deny access to therapies.

Figure 1:. Summary of the primary recommendations for the initial and subsequent treatment of children with JIA and active polyarthritis (see also Tables 3 and 4; for patients with sacroiliitis and/or enthesitis, see also Tables 5 and 6).

PICO questions in brackets, quality of evidence in parentheses. Strength of recommendation indicated by colors (see legend). The clinical Juvenile Arthritis Disease Activity Score based on 10 joints (cJADAS-10) was used to define low disease activity (≤ 2.5 with ≥ 1 active joint) versus high/moderate disease activity (> 2.5). While this is provided as a general parameter, the cJADAS-10 should be interpreted within the clinical context. An adequate trial of methotrexate was considered to be 3 months. If no or minimal response is observed after 6–8 weeks, it was agreed that changing or adding therapy may be appropriate. Shared decision-making between the physician, parents, and patient, including discussion of recommended treatments and potential alternatives, is recommended when initiating or escalating treatment. * DMARD over biologic recommended for patients without and with risk factors, although initial biologic therapy may be appropriate for some patients with risk factors and involvement of high risk joints, high disease activity, and/or those judged by their physician to be at high risk of disabling joint damage. ** Adding a biologic may be considered in biologic naïve patients with continued low disease activity after escalating therapy (not formally addressed in the guidelines) DMARD = non-biologic disease modifying anti-rheumatic drug (methotrexate, leflunomide or sulfasalazine). TNFi = tumor necrosis factor inhibitor, triple DMARD therapy = methotrexate, sulfasalazine, and hydroxychloroquine, NSAID = non-steroidal anti-inflammatory drug, PT = physical therapy, OT = occupational therapy.