Guideline sources The following summarized guidelines for the evaluation and management of juvenile idiopathic arthritis are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023; 2022; 2019; 2013), the European League Against Rheumatism (EULAR 2022), the Royal College of Ophthalmologists (RCOphth 2020), the German Society of Pediatric Rheumatology (GKJR/BVA/DGRh/DOG 2019), the Arthritis Foundation (AF/ACR 2019), the Ministry of Health, Labour and Welfare of Japan (MHLW 2018), and the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2018). 1 2 3 4 5 6 7 8 9 10 11 Guidelines 1. Diagnostic investigations Diagnostic imaging: do not obtain radiography as a screening test before advanced imaging for the purpose of identifying active synovitis or enthesitis. D Ophthalmic screening: As per ACR 2019 guidelines: https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 1/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway Consider obtaining ophthalmic screening every 3 months in pediatric and adolescent patients with JIA at high risk of developing uveitis. C Educate pediatric and adolescent patients with spondyloarthritis regarding the warning signs of acute anterior uveitis for the purpose of decreasing the delay in treatment, duration of symptoms, or complications of iritis. B Pretreatment evaluation: As per ACR 2022 guidelines: Consider obtaining baseline laboratory testing before initiating pharmacotherapy. Consider screening for tuberculosis before initiating biologic DMARD therapy and when there is a concern for tuberculosis exposure thereafter. C As per EULAR 2022 guidelines, screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 2. Medical management Management of oligoarticular JIA (NSAIDs): consider initiating a trial of scheduled NSAIDs as part of initial therapy in patients with active oligoarticular JIA. C Management of oligoarticular JIA (corticosteroids): avoid using oral corticosteroids as part of initial therapy in patients with active oligoarticular JIA. D Management of oligoarticular JIA (DMARDs and biologics): Initiate conventional synthetic DMARDs in patients with active oligoarticular JIA with an inadequate response to scheduled NSAIDs and/or intra-articular corticosteroids. Consider preferring methotrexate B over leflunomide, sulfasalazine or hydroxychloroquine. B Initiate biologic DMARDs in patients with active oligoarticular JIA with an inadequate response to or intolerance of NSAIDs and/or intra-articular corticosteroids and at least one conventional synthetic DMARD. Insufficient evidence to recommend a preferred biologic DMARD. B Management of oligoarticular JIA (treatment monitoring): Consider taking into account the following risk factors for poor outcome to guide treatment decisions: involvement of ankle, wrist, hip, sacroiliac joint and/or TMJ presence of erosive disease or enthesitis delay in diagnosis elevated levels of inflammatory markers symmetric disease. C Consider using validated disease activity measures to guide treatment decisions, especially to facilitate treat-to-target approaches. C https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 2/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway Management of polyarticular JIA (NSAIDs): consider initiating NSAIDs as adjunct therapy in patients with polyarticular JIA. C Management of polyarticular JIA (corticosteroids): consider offering bridging therapy with a limited course of oral corticosteroids (< 3 months) during initiation or escalation of therapy in patients with polyarticular JIA with high or moderate disease activity (cJADAS-10 > 2.5). C Show 2 more Management of polyarticular JIA (DMARDs): initiate DMARDs over NSAID monotherapy as initial therapy in patients with polyarticular JIA. B Show 6 more Management of polyarticular JIA (biologics): consider adding a biologic to the original DMARD over changing to a second DMARD in patients with polyarticular JIA and moderate or high disease activity despite DMARD monotherapy. C Show 5 more Management of systemic-onset JIA, NSAIDs: As per ACR 2022 guidelines, consider initiating NSAIDs as initial monotherapy in patients with systemic-onset JIA without macrophage activation syndrome. C As per ACR 2013 guidelines, initiate NSAID monotherapy as a treatment option in patients without prior treatment and with an MD global score < 5, irrespective of the active joint count. B Show 4 more Management of systemic-onset JIA, corticosteroids: As per ACR 2022 guidelines, avoid using oral corticosteroids as initial monotherapy in patients with systemic-onset JIA without macrophage activation syndrome. D Show 2 more As per ACR 2013 guidelines, initiate systemic corticosteroid monotherapy (PO or IV) for a maximum period of 2 weeks as a therapeutic option in patients with: an MD global score < 5 and an active joint count > 4 an MD global score 5, irrespective of the active joint count. B Show 5 more Management of systemic-onset JIA, DMARDs: As per ACR 2022 guidelines: Do not use conventional synthetic DMARDs as initial monotherapy in patients with systemic- onset JIA without macrophage activation syndrome. D Initiate conventional synthetic or biologic synthetic DMARDs over long-term corticosteroids in patients with residual arthritis and incomplete response to IL-1 and/or IL-6 inhibitors, in patients with or without macrophage activation syndrome. Insufficient evidence to recommend a preferred agent. B As per ACR 2013 guidelines, initiate methotrexate or leflunomide in patients with an MD global score < 5 and an active joint count > 0 after treatment with corticosteroid monotherapy B , an IL-1 inhibitor, or tocilizumab. B https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 3/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway Show 9 more Management of systemic-onset JIA, biologics: As per ACR 2022 guidelines, consider initiating biologic DMARDs (IL-1 or IL-6 inhibitors) as initial monotherapy in patients with systemic-onset JIA without macrophage activation syndrome. Insufficient evidence to recommend a preferred agent. C Show 3 more As per ACR 2013 guidelines, initiate anakinra as an initial therapeutic option in patients with an MD global score 5, irrespective of the active joint count, or an MD global score < 5 and an active joint count > 0. B Show 25 more Management of systemic-onset JIA (IVIg): Do not use IVIG in patients with continued disease activity, irrespective of the active joint count and MD global score. D Insufficient evidence to support the use of IVIG in patients tried a calcineurin inhibitor in combination with anakinra. I Management of sacroiliitis: initiate NSAIDs in pediatric and adolescent patients with JIA and active sacroiliitis. B Show 6 more Management of enthesitis: initiate NSAIDs in pediatric and adolescent patients with JIA and active enthesitis. B Show 3 more Management of TMJ arthritis: consider initiating a trial of scheduled NSAIDs as part of initial therapy in patients with active TMJ arthritis. C Show 4 more Management of uveitis, general principles: As per BVA/GKJR/DGRh/DOG 2019 guidelines, initiate treatment in patients with JIA-associated active uveitis. E As per SHARE initiative 2018 guidelines, initiate immediate treatment in patients with JIA- associated active uveitis. B Show 2 more Management of uveitis, topical corticosteroids: As per AF/ACR 2019 guidelines, consider offering prednisolone acetate 1% topical drops over difluprednate topical drops in pediatric and adolescent patients with JIA and active chronic anterior uveitis. C Show 6 more As per BVA/GKJR/DGRh/DOG 2019 guidelines: Offer topical corticosteroids as initial treatment in patients with JIA-associated active uveitis. E https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 4/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway Offer topical corticosteroids at the lowest dose depending on the individual course in patients with JIA-associated chronic uveitis. Do not initiate systemic DMARDs if inactivity on a maintenance dosage of 2 drops daily is achieved within 3 months. E As per SHARE initiative 2018 guidelines, offer topical corticosteroids (preferably prednisolone acetate or dexamethasone) as first-line therapy in patients with JIA-associated anterior uveitis. B Management of uveitis, conventional DMARDs: As per AF/ACR 2019 guidelines, consider administering methotrexate subcutaneous over oral methotrexate in pediatric and adolescent patients with JIA and chronic anterior uveitis starting systemic treatment for uveitis. C Show 6 more As per BVA/GKJR/DGRh/DOG 2019 guidelines, initiate conventional synthetic DMARDs in patients with JIA-associated uveitis if disease inactivity is not achieved by 12 weeks of topical corticosteroid treatment of 2 drops daily, or if the topical corticosteroids result in adverse effects, or if new, inflammation-related complications of uveitis occur. Consider decreasing the interval in patients with very severe uveitis. E As per SHARE initiative 2018 guidelines, initiate systemic immunosuppression in patients with JIA-associated active uveitis with poor prognostic factors identified at the first visit or during the disease course. B Show 2 more Management of uveitis, biological DMARDs: As per AF/ACR 2019 guidelines, consider initiating methotrexate and a monoclonal antibody TNF inhibitor immediately in pediatric and adolescent patients with JIA-associated severe active chronic anterior uveitis and sight-threatening complications. C Show 4 more As per BVA/GKJR/DGRh/DOG 2019 guidelines: Initiate TNF-alpha antibody therapy in combination with methotrexate if uveitis inactivity is not achieved by 16 weeks of methotrexate monotherapy and 2 drops of topical corticosteroids daily, or if new, inflammation-related complications of uveitis occur. Consider initiating this escalated treatment at shorter intervals in patients with very severe uveitis. E Switch to another biological agent if TNF-alpha antibodies fail to take effect or lose effect. E As per SHARE initiative 2018 guidelines, add or switch to biological treatment in case of methotrexate inefficacy or intolerance. Initiate anti-TNF agents (adalimumab > infliximab > golimumab) in patients with uveitis refractory/resistant to DMARD therapy, principally methotrexate. Consider switching between different anti-TNF treatments if uveitis is refractory to the first anti-TNF. B Show 3 more Management of uveitis (systemic corticosteroids): consider administering systemic corticosteroids for bridging until conventional or biological DMARDs take effect in patients with very severe active uveitis with prognostic risk factors for impending uveitis-related deterioration of vision (such as hypotony, high Tyndall effect, massive anterior chamber inflammation, dense https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 5/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway vitreous opacities, or macular edema) potentially causing further worsening of vision in the short term. E Management of uveitis (ocular corticosteroid injections): consider administering ocular corticosteroid injections as rescue therapy in individual patients with unilateral or bilateral severe active uveitis with prognostic risk factors for impending uveitis-related deterioration of vision (such as hypotony, macular edema, or dense vitreous opacities), after exhaustion of all other treatment options. E Management of uveitis, NSAIDs: As per BVA/GKJR/DGRh/DOG 2019 guidelines, do not use topical and systemic NSAIDs alone for the treatment of patients with JIA-associated active uveitis. D As per SHARE initiative 2018 guidelines, do not use topical and systemic NSAIDs as monotherapy in patients with JIA-associated uveitis, but consider prescribing NSAIDs as additional therapy. D Management of uveitis (cycloplegics): administer cycloplegics to prevent or treat posterior synechiae in patients with active uveitis. E Management of uveitis, assessment of treatment response: As per AF/ACR 2019 guidelines, obtain ophthalmic monitoring within 1 month after each change of topical corticosteroids in pediatric and adolescent patients with JIA and controlled uveitis tapering or discontinuing topical corticosteroids. B Show 2 more As per SHARE initiative 2018 guidelines, ensure good communication between the ophthalmologist and the pediatric rheumatologist concerning changes in disease activity treatment changes and responsibility for treatment monitoring. B Management of uveitis, treatment tapering: As per AF/ACR 2019 guidelines, consider tapering treatment after at least 2 years of well- controlled disease on DMARDs and biologic systemic therapy only. C As per BVA/GKJR/DGRh/DOG 2019 guidelines, de-escalate DMARD therapy after at least 2 years of uveitis inactivity. E As per SHARE initiative 2018 guidelines, taper systemic immunosuppression (both DMARDs and biological therapies) after 2 years of inactive disease of topical corticosteroids. B 3. Nonpharmacologic interventions Dietary modifications: counsel patients regarding a healthy, age-appropriate diet. B Show 2 more Physical and occupational therapy: Consider offering physical and occupational therapy regardless of concomitant pharmacotherapy. C https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 6/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway Consider offering physical therapy in pediatric and adolescent patients with polyarthritis, sacroiliitis or enthesitis at risk for functional limitations. C 4. Therapeutic procedures Intra-articular corticosteroids (oligoarticular JIA): Administer intra-articular corticosteroids as part of initial therapy in patients with active oligoarticular JIA. B Prefer triamcinolone hexacetonide over triamcinolone acetonide for intra-articular corticosteroid injections. B Intra-articular corticosteroids (polyarticular JIA): Consider administering intra-articular corticosteroids as adjunct therapy in patients with polyarticular JIA. C Prefer triamcinolone hexacetonide over triamcinolone acetonide for intra-articular corticosteroid injections. B Intra-articular corticosteroids (systemic-onset JIA): Consider administering ntra-articular corticosteroid injection as an initial treatment option in patients with systemic-onset JIA with an active joint count 4. C Consider administering intra-articular corticosteroid injection as an adjunct therapy at any time in patients with systemic-onset JIA. C Intra-articular corticosteroids (sacroiliitis): consider administering intra-articular corticosteroid injection of the sacroiliac joints as adjunct therapy in pediatric and adolescent patients with active sacroiliitis despite treatment with NSAIDs. C Intra-articular corticosteroids (TMJ arthritis): consider administering intra-articular corticosteroids as part of initial therapy in patients with active TMJ arthritis. Insufficient evidence to recommend a preferred agent. C Intra-articular corticosteroids (imaging guidance): consider obtaining imaging guidance during intra-articular corticosteroid injections in joints difficult to access, or to specifically localize the distribution of inflammation. C 5. Preventative measures Routine immunizations: As per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 7/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway As per ACR 2022 guidelines, provide annual inactivated influenza immunization in all pediatric patients with JIA. B Show 5 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C 6. Follow-up and surveillance Treatment monitoring (NSAIDs): consider obtaining a CBC, LFTs and renal function tests every 6-12 months for monitoring of patients taking NSAIDs. C Treatment monitoring (DMARDs): obtain CBC, LFTs and renal function tests within the first 1-2 months of usage and every 3-4 months thereafter for monitoring of patients taking methotrexate. B Show 7 more Treatment monitoring (hydroxychloroquine/chloroquine): obtain annual monitoring for retinopathy in all patients receiving hydroxychloroquine for > 5 years or chloroquine for > 1 year. B Show 17 more Treatment monitoring (biologics): consider obtaining CBC and LFTs annually for monitoring of patients taking TNF inhibitors. C Show 5 more Clinical findings Symptoms Anthropomorphic exam Abdominal pain Growth restriction Chest pain Head and neck exam Difficulty opening mouth Fatigue Micrognathia Fever Retrognathia Irritability Respiratory exam Jaw pain Joint pain Pleuritis Joint swelling Loss of appetite Musculoskeletal exam Skin rash Arthritis Weight loss Limping Ocular exam https://web.pathway.md/diseases/recJFMuKYxmWTbSKR 8/10 6/29/23, 4:07 AM Juvenile idiopathic arthritis Pathway Anterior uveitis Lab findings Cardiac exam ESR serum ALT Pericarditis serum AST serum CRP Abdominal exam serum IgG Hepatomegaly serum LDH Splenomegaly serum ferritin ESR Lymphatic exam serum fibrinogen Axillary lymphadenopathy Serological findings Epitrochlear lymphadenopathy RF Hematological findings anti-citrullinated cyclic peptide antibodies Anemia anti-neutrophil cystoplasmic antibodies Coagulopathy Pancytopenia WBC count blood neutrophil count blood platelet count References 1. 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