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Today we’ll be going over the American College of Rheumatology’s newest guideline on Juvenile Idiopathic Arthritis. 

The purpose of this guideline is to promote early and aggressive treatment of juvenile idiopathic arthritis (JIA) to preserve function and maximize outcomes and quality of life; encourage timely screening and monitoring to prevent articular and extra-articular damage; and facilitate effective shared decision making among clinicians, patients, and caregivers.

In today’s rapid update, we’ll just be going over a summary of key recommendations so for the full guideline and recommendations, make sure to check it out on guidelinecentral.com

Let’s get started. 

Starting with the section on Treatment of Systemic Juvenile Idiopathic Recommendations

In people with newly diagnosed sJIA without MAS,

  • The guideline strongly recommends bDMARD therapies as first-line treatment.
  • The guideline strongly recommends against NSAIDs as initial monotherapy.
  • The guideline conditionally recommends against oral glucocorticoids as initial monotherapy.
  • The guideline strongly recommends against csDMARDs as initial monotherapy

In people with sJIA without MAS with ongoing systemic symptoms who do not respond to or are intolerant to initial therapy with a bDMARD:

  • The guideline conditionally recommends a different bDMARD or targeted synthetic DMARD over the addition of a csDMARD or glucocorticoids.

In people with sJIA without MAS with well-controlled systemic symptoms but residual arthritis on bDMARD

  • The guideline conditionally recommends changing to a different bDMARD or tsDMARD, adding csDsMARD, or intraarticular glucocorticoid injection, over the addition of systemic glucocorticoids.

In people with newly diagnosed sJIA with MAS

  • The guideline strongly recommends bDMARD therapies as first-line treatment.
  • The guideline strongly recommends systemic glucocorticoids as part of initial therapy.

In people with sJIA with active MAS who do not respond to or are intolerant to initial therapy with a bDMARD

  • The guideline conditionally recommends a different bDMARD or tsDMARD over addition of a csDMARD.

In people with inactive sJIA with or without history of MAS

  • The guideline strongly recommends tapering and discontinuing glucocorticoids after clinical inactive disease has been attained for sJIA.
  • The guideline conditionally recommends tapering and discontinuing bDMARDs over immediately stopping bDMARDs after CID has been attained for sJIA.

In people with sJIA with or without a history of MAS who have achieved CID on DMARDs

  • No consensus could be reached regarding a specific length of time in CID before tapering or stopping DMARD

In people with sJIA with or without a history of MAS who have flared upon DMARD taper or discontinuation

  • The guideline conditionally recommends restarting the same medication regimen as the most recently effective regimen over starting a new regimen to recapture inactive disease and remission after flare

In people with sJIA

  • The guideline conditionally recommends routine screening for sJIA-LD.
  • The guideline strongly recommends that the presence or development of lung disease should not be considered an absolute contraindication to the use of IL-1/6 inhibitors

Next the section on Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Polyarthritis, Oligoarthritis, Enthesitis, Dactylitis, Temporomandibular Joint Arthritis, and Deprescribing Recommendations

In people with non-systemic JIA

  • Risk factors for poor outcomes should be considered in non-systemic JIA to guide treatment decisions.
  • The guideline conditionally recommends use of validated JIA disease activity measures to guide treatment decisions for non-systemic JIA, especially to facilitate treat-to-target approaches

In people with polyarthritis

  • The guideline conditionally recommends a trial of scheduled oral NSAIDs as adjuncts to initial therapy.
  • The guideline conditionally recommends intra-articular glucocorticoids as adjuncts to initial therapy. 
  • The guideline strongly recommends triamcinolone hexacetonide as the preferred agent for IAGC injections. 
  • The guideline conditionally recommends against oral glucocorticoids as adjuncts to initial therapy. 
  • The guideline strongly recommends DMARDs as firstline therapy. 
  • The guideline conditionally recommends methotrexate as the first csDMARD over leflunomide, sulfasalazine, or hydroxychloroquine. 
  • The guideline conditionally recommends oral methotrexate over subcutaneous methotrexate. 
  • The guideline conditionally recommends tumor necrosis factor inhibitors as the first bDMARD over the following bDMARDS: IL-6i, T cell costimulation modulators, IL-17i, IL-12/23i

In people with polyarthritis treated with TNFi

  • The guideline conditionally recommends using a csDMARD concurrently.

In people with polyarthritis treated with bDMARDs

  • The guideline conditionally recommends against routinely monitoring anti-drug antibodies.

In people with oligoarthritis

  • The guideline conditionally recommends a trial of scheduled oral NSAIDs as first-line therapy. 
  • The guideline strongly recommends IAGCs as first-line therapy. 
  • The guideline strongly recommends triamcinolone hexacetonide as the preferred agent for IAGC injections. 
  • The guideline conditionally recommends against oral glucocorticoids as adjuncts to initial therapy. 
  • The guideline strongly recommends csDMARDs over no csDMARDs for an inadequate response to scheduled NSAIDs and/or IAGCs. 
  • The guideline conditionally recommends methotrexate as the first csDMARD over leflunomide, sulfasalazine, or hydroxychloroquine.
  • The guideline conditionally recommends oral methotrexate over subcutaneous methotrexate. 
  • The guideline strongly recommends bDMARDs for an inadequate response to or intolerance of NSAIDs and/or IAGCs and first-line csDMARD. 
  • The guideline conditionally recommends TNFi as the first bDMARD over the following bDMARDs: IL-6i, T cell costimulation-modulators, IL-17i, IL-12/23i.

In people with oligoarthritis treated with TNFi

  • The guideline conditionally recommends using a csDMARD concurrently. 

In people with oligoarthritis treated with biologic bDMARDs

  • The guideline conditionally recommends against monitoring ADA levels routinely. 

In people with enthesitis

  • The guideline conditionally recommends a trial of scheduled oral NSAIDs as adjuncts to initial therapy. 
  • The guideline conditionally recommends against oral glucocorticoids as adjuncts to initial therapy. 
  • The guideline strongly recommends DMARDs as firstline therapy. 
  • The guideline conditionally recommends methotrexate as the first csDMARD over sulfasalazine. 
  • The guideline conditionally recommends oral methotrexate over subcutaneous methotrexate. 
  • The guideline conditionally recommends TNFi as the first bDMARD over the following bDMARDs: IL-17i, IL-12/23i.

In people with enthesitis treated with TNFi

  • The guideline conditionally recommends using a csDMARD concurrently

In people with enthesitis treated with bDMARDs

  • The guideline conditionally recommends against routinely monitoring anti-drug antibodies.

In people with dactylitis

  • The guideline conditionally recommends a trial of scheduled oral NSAIDs as adjuncts to initial therapy. 
  • The guideline strongly recommends IAGCs and tendon injections as adjuncts to initial therapy.  
  • There is no preferred agent for IAGC. 
  • The guideline conditionally recommends against oral glucocorticoids as adjuncts to initial therapy. 
  • The guideline strongly recommends DMARDs as firstline therapy. 
  • The guideline conditionally recommends methotrexate as the first csDMARD over leflunomide, sulfasalazine, or hydroxychloroquine. 
  • The guideline conditionally recommends oral methotrexate over subcutaneous methotrexate. 
  • The guideline conditionally recommends TNFi as the first bDMARD over the following bDMARDs: IL-17i, IL-12/23i.

In people with dactylitis treated with TNFi

  • The guideline conditionally recommends using a csDMARD concurrently. 

In people with dactylitis treated with bDMARD 

  • The guideline conditionally recommends against routinely monitoring anti-drug antibodies

In people with TMJ arthritis 

  • The guideline conditionally recommends a trial of scheduled oral NSAIDs as adjuncts to initial therapy. 
  • The guideline conditionally recommends IAGCs as adjuncts to initial therapy. No agent is preferred. 
  • The guideline conditionally recommends against oral glucocorticoids as adjuncts to initial therapy.
  • The guideline strongly recommends DMARDs as firstline therapy. 
  • The guideline conditionally recommends methotrexate as the first csDMARD over leflunomide. 
  • The guideline conditionally recommends oral methotrexate over subcutaneous methotrexate. 
  • The guideline conditionally recommends TNFI as the first bDMARD over the following bDMARDs: IL-6i, T cell costimulation-modulators, IL-17i, IL-12/23i.  

In people with TMJ arthritis taking TNFi 

  • The guideline conditionally recommends using a csDMARD concurrently.  

In people with TMJ arthritis treated with bDMARDs 

  • The guideline conditionally recommends against routinely monitoring anti-drug antibodies.

In people with JIA with inadequate response or intolerance to first-line csDMARD 

  • The guideline strongly recommends bDMARDS. 

 In people with JIA with inadequate response or intolerance to first-line TNFi 

  • The guideline conditionally recommends increasing the dose of first TNFi, changing to a second TNFi or using a medication with a different mechanism of action. No strategy is preferred.  

In people with non-systemic JIA in clinical remission on bDMARDs 

  • The guideline conditionally recommends tapering bDMARDs over immediately stopping bDMARDs to prevent disease exacerbation.  

In people with non-systemic JIA in clinical remission on combination DMARDs 

  • The guideline conditionally recommends tapering or stopping csDMARDs first over tapering or stopping bDMARds or tsDMARDs.  

In people with non-systemic JIA in clinical remission on DMARDs 

  • The guideline conditionally recommends imaging joints that are difficult to assess over not performing imaging when considering tapering or stopping medication.  

In people with non-systemic JIA with flare after DMARD deprescribing 

  • The guideline conditionally recommends against IAGCs over restarting DMARDs to recapture inactive disease. 
  • The guideline conditionally recommends restarting the most recently effective DMARD regimen over starting a new regimen to recapture inactive disease.

Then on to the section on Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Management, Medication Monitoring, and Imaging Recommendations

In people with JIA 

  • The guideline conditionally recommends screening for mental health concerns whenever possible and referral for appropriate treatment.  
  • The guideline conditionally recommends PT and OT regardless of concomitant pharmacologic therapy. 
  • The guideline conditionally recommends physical activity regardless of concomitant pharmacologic therapy. 
  • The guideline strongly recommends against use of a specific diet alone to treat JIA; however, a discussion of healthy, age-appropriate diet is encouraged. 
  • The guideline conditionally recommends against use of supplemental or herbal interventions specifically to treat JIA. 
  • The guideline strongly recommends assessment for transition readiness and creation of a transition plan.

In people with JIA 

  • The guideline conditionally recommends baseline laboratory testing prior to treatment initiation for all medications.  

In people with JIA receiving chronic treatment with oral or intravenous glucocorticoids 

  • The guideline strongly recommends monitoring for dyslipidemia, hyperglycemia, bone and ocular health at least annually. 
  • The guideline strongly recommends monitoring growth and blood pressure at least twice a year.

In people with JIA receiving treatment with NSAIDS 

  • The guideline conditionally recommends monitoring via CBCD, LFTs, and renal function tests every 6-12 months.

In people with JIA receiving treatment with methotrexate 

  • The guideline strongly recommends monitoring via CBCD, LFTs, and renal function tests within the first 1-2 months of usage and every 3-4 months thereafter. 
  • The guideline conditionally recommends altering methotrexate administration if a clinically relevant elevation in liver enzymes occurs. 
  • The guideline strongly recommends using folic/folinic acid in conjunction with methotrexate.

In people with JIA receiving treatment with sulfasalazine 

  • The guideline conditionally recommends monitoring via CBCD, LFTs, and renal function tests within the first 1-2 months of usage and every 3-4 months thereafter. 
  • The guideline conditionally recommends decreasing the sulfasalazine dosage or holding sulfasalazine if a clinically relevant elevation in liver enzymes or decreased neutrophil or platelet count is found.

In people with JIA receiving treatment with leflunomide 

  • The guideline conditionally recommends monitoring via CBCD and LFT’s within the first 1-2 months of usage and every 3-4 months thereafter. 
  • The guideline conditionally recommends altering leflunomide administration if a clinically relevant elevation in liver enzymes occurs.

In people with JIA receiving treatment with hydroxychloroquine 

  • The guideline conditionally recommends monitoring via CBCD and LFTs annually. 
  • The guideline conditionally recommends baseline and annual retinal screening after starting hydroxychloroquine. 

In people with JIA receiving treatment with calcineurin inhibitors 

  • The guideline conditionally recommends monitoring CBCD, uric acid, liver enzymes, electrolytes, magnesium, renal function, and blood pressure 4-8 weeks after starting treatment and then every 1-3 months thereafter. 
  • The guideline conditionally recommends monitoring lipids at baseline, then 4 weeks after starting treatment and every 6 months thereafter. 
  • The guideline conditionally recommends reducing the dose by 50% administration if serum creatinine increases by more than 50%. Drug should be discontinued if serum creatinine does not improve.  

In people with JIA receiving treatment with mycophenolate 

  • The guideline conditionally recommends monitoring via CBCD and LFTs within the first 1 month of usage and every 3 months thereafter.  

In people with JIA receiving treatment with tumor necrosis factor inhibitors 

  • The guideline conditionally recommends monitoring via CBCD and LFTs annually.  

In people with JIA receiving treatment with abatacept 

  • The guideline conditionally recommends doing no routine laboratory monitoring. 

In people with JIA receiving treatment with interleukin-6 inhibitors 

  • The guideline conditionally recommends monitoring via CBCD and LFTs within the first 1-2 months of usage and every 3-4 months thereafter. 
  • The guideline conditionally recommends altering IL-6i administration if monitoring reveals elevated liver enzymes, neutropenia, or thrombocytopenia as per package insert. 
  • The guideline conditionally recommends monitoring of lipid levels every 6 months. 

And last the section on Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis Recommendations

In children and adolescents with JIA at high risk of developing CAU 

  • The guideline strongly recommends ophthalmic screening every 3 months as compared to monitoring less frequently.

In children and adolescents with JIA and controlled CAU on stable therapy 

  • The guideline strongly recommends ophthalmic monitoring no less frequently than every 3 months as compared to monitoring less frequently.  

In children and adolescents with JIA and controlled CAU who are tapering or stopping glucocorticoids

  •  The guideline strongly recommends ophthalmic monitoring within one month after each change of topical glucocorticoids as compared to monitoring less frequently.  

In children and adolescents with JIA and controlled CAU who are tapering or discontinuing DMARDs 

  • The guideline strongly recommends ophthalmic monitoring within two months of changing DMARD therapy as compared to monitoring less frequently.

In children and adolescents with JIA and active CAU 

  • The guideline conditionally recommends adding or increasing topical prednisolone acetate 1% for short-term control over adding systemic glucocorticoids. The guideline conditionally recommends using prednisolone acetate 1% topical drops over difluprednate topical drops.  

In children with JIA with CAU, irrespective of use of or DMARD therapy 

  • The guideline conditionally recommends against intraocular and periocular glucocorticoid injections as part of therapy.  

In children and adolescents with JIA who develop new CAU activity 

  • The guideline conditionally recommends adding or increasing topical glucocorticoids only for short-term control over changing/escalating DMARD therapy immediately.  

In children and adolescents with JIA and CAU still requiring 1-2 drops/day of prednisolone acetate 1% for CAU control, and not on DMARD therapy 

  • The guideline conditionally recommends adding DMARD therapy in order to taper topical glucocorticoids over not adding DMARD therapy and maintaining on topical glucocorticoids alone.  

In children and adolescents with JIA and CAU still requiring 1-2 drops/day of prednisolone acetate 1% for at least 3 months and on DMARD therapy for CAU control 

  • The guideline conditionally recommends changing or escalating DMARD therapy over maintaining current DMARD therapy.

In children and adolescents with JIA and active CAU requiring 1-2 drops/day of prednisolone acetate 1% 

  • The guideline conditionally recommends DMARDS when a patient is newly diagnosed with JIA-associated CAU.  

In children and adolescents with JIA and CAU 

  • The guideline conditionally recommends methotrexate over leflunomide, mycophenolate, and cyclosporine.  

In children and adolescents with JIA and CAU who are starting DMARD treatment for CAU 

  • The guideline conditionally recommends using oral methotrexate over subcutaneous methotrexate.  

In children and adolescents with JIA and CAU 

  • The guideline conditionally recommends adalimumab as a DMARD over infliximab, tocilizumab, and either golimumab, abatacept, Janus kinase inhibitors, or rituximab.

In children and adolescents with JIA with severe active CAU and sight-threatening complications 

  • The guideline conditionally recommends starting a csDMARD and a bDMARD at treatment onset over csDMARD monotherapy.  

For initial treatment in children and adolescents with JIA with active CAU regardless of joint activity 

  • The guideline conditionally recommends using above-standard JIA dosing of TNFi over standard JIA dosing for TNFi.

In children and adolescents with JIA and active CAU who have an inadequate response to one monoclonal antibody TNFi at standard JIA dose 

  • The guideline conditionally recommends escalating the dose and/or frequency to above-standard vs. switching to another monoclonal antibody TNFi.  

In children with JIA and CAU, requiring 1-2 drops/day of prednisolone acetate 1%  for at least 3 months despite csDMARD and two successive TNFi monoclonal antibodies 

  • The guideline conditionally recommends a different csDMARD, non-TNFi bDMARD, or tsDMARD therapy.

In children and adolescents with JIA and CAU that is controlled on DMARD therapy but remain on 1-2 drops/day of prednisolone acetate 1% 

  • The guideline conditionally recommends tapering topical glucocorticoids first over tapering DMARD therapy.  

In children and adolescents with CAU that is well controlled on DMARD only 

  • The guideline conditionally recommends that there be at least 2 years of well-controlled disease before tapering therapy.

And there you have it. Make sure to check out the full guideline from the American College of Rheumatology and other related clinical decision support tools at guidelinecentral.com.

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