Treatment of JIA-ERA (Enthesitis-Related Arthritis)
For children with JIA-ERA, sulfasalazine is the preferred first-line DMARD following NSAIDs and/or intraarticular glucocorticoid injections, with TNF inhibitors reserved for inadequate response to sulfasalazine. 1
Initial Therapy Approach
NSAIDs as First-Line
- NSAIDs are conditionally recommended as adjunct therapy for pain and inflammation in JIA-ERA 1
- Naproxen is the preferred NSAID over other COX-1 or COX-2 inhibitors due to its efficacy and safety profile in children 1
- An adequate trial period of at least 8 weeks is required, though response typically occurs within 1 month 1
- NSAID initiation should not delay introduction of DMARD therapy 1
- Alternative NSAIDs (ibuprofen, indomethacin, meloxicam) can be used if naproxen is contraindicated or unavailable 1
Intraarticular Glucocorticoid Injections
- Intraarticular glucocorticoid injections are conditionally recommended as adjunct therapy for all patients with active arthritis 1
- Triamcinolone hexacetonide is strongly recommended over triamcinolone acetonide 1
- Expected to provide clinical improvement for at least 4 months; shorter duration suggests need for systemic therapy escalation 1
DMARD Therapy for ERA
Sulfasalazine as Preferred First-Line DMARD
- Sulfasalazine is specifically recommended for enthesitis-related arthritis following glucocorticoid joint injection or adequate NSAID trial in patients with moderate or high disease activity 1
- This recommendation is based on moderate-level evidence specifically for the ERA subtype 1
- Sulfasalazine can be initiated without prior methotrexate in ERA patients 1
Methotrexate as Alternative
- Methotrexate remains an option if sulfasalazine is contraindicated or not tolerated 1
- Subcutaneous methotrexate is conditionally recommended over oral formulation for better bioavailability 1, 2
- Doses of 10-15 mg/m² BSA per week are recommended; doses above 15 mg/m² BSA per week show no additional benefit 1
Biologic DMARD Therapy
TNF Inhibitors for Refractory Disease
- TNF inhibitors are recommended for ERA patients who have received glucocorticoid joint injections and an adequate trial of sulfasalazine (without prior methotrexate) with moderate or high disease activity 1
- This recommendation applies irrespective of prognostic features 1
- FDA-approved TNF inhibitors for JIA include etanercept (ages ≥2 years) and adalimumab (ages ≥2 years) 3, 4, 3
- Combination therapy with a DMARD is conditionally recommended for TNF inhibitors (except infliximab, which strongly requires combination therapy) 1
Disease Activity Monitoring
Treatment Targets
- Use validated disease activity measures (JADAS-27) to guide treatment decisions 5, 2
- Disease activity categories: inactive, low (JADAS-27 1.1-≤8.5), moderate, or high 1, 2
- An adequate trial of DMARD therapy is 3 months, though escalation may be appropriate after 6-8 weeks if minimal response 1
Critical Pitfalls to Avoid
Common Errors in ERA Management
- Do not use methotrexate as automatic first-line in ERA - sulfasalazine has specific evidence for this subtype 1
- Do not continue NSAID monotherapy beyond 2 months in patients with active arthritis 1
- Strongly recommend against chronic low-dose oral glucocorticoids irrespective of disease activity 1
- Do not delay DMARD initiation while waiting for NSAID response 1
Safety Monitoring
- Screen for tuberculosis before initiating TNF inhibitors and periodically during therapy 3, 4
- Complete age-appropriate vaccinations before starting biologic therapy 3
- Monitor for serious infections, which are the primary safety concern with TNF inhibitors 3, 4
Treatment Algorithm Summary
- Start with NSAIDs (preferably naproxen) + intraarticular glucocorticoids for symptomatic relief 1
- Initiate sulfasalazine if moderate/high disease activity or inadequate response after 2 months 1
- Add TNF inhibitor if inadequate response to sulfasalazine after adequate trial 1
- Consider switching to non-TNF biologic if inadequate response to first TNF inhibitor 1