Guideline sources The following summarized guidelines for the evaluation and management of juvenile idiopathic arthritis-associated uveitis are prepared by our editorial team based on guidelines from the German Society of Pediatric Rheumatology (GKJR/BVA/DGRh/DOG 2019), the Arthritis Foundation (AF/ACR 2019), and the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2018). 1 2 3 Guidelines 1. Screening and diagnosis Indications for screening: As per ACR 2019 guidelines, consider obtaining ophthalmic screening every 3 months in pediatric and adolescent patients with JIA at high risk of developing uveitis. C As per SHARE initiative 2018 guidelines, obtain screening for uveitis according to a contemporary and audited protocol in all patients with JIA. Implement formal screening protocols in all centers, where patients with JIA are seen. B https://web.pathway.md/diseases/recNlaJhQNTnqs8AH 1/5 6/29/23, 4:07 AM Juvenile idiopathic arthritis-associated uveitis Pathway Show 2 more 2. Classification and risk stratification Severity assessment: insufficient evidence to recommend a validated biomarker to follow the activity of uveitis. I 3. Medical management General principles: As per 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 Topical corticosteroids: As per ACR 2019 guidelines, consider offering prednisolone acetate 1% topical drops over difluprednate topical drops in pediatric and adolescent patients with JIA-associated active chronic anterior uveitis. C Show 4 more As per DOG 2019 guidelines: Offer topical corticosteroids as initial treatment in patients with JIA-associated active uveitis. E 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 Disease-modifying antirheumatic drugs, conventional DMARDs: As per AF/ACR 2019 guidelines, consider adding systemic therapy to taper topical corticosteroids in pediatric and adolescent patients with JIA-associated chronic anterior uveitis still requiring 1-2 drops/day of prednisolone acetate 1% (or equivalent) for uveitis control, and not on systemic therapy. C Show 2 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 https://web.pathway.md/diseases/recNlaJhQNTnqs8AH 2/5 6/29/23, 4:07 AM Juvenile idiopathic arthritis-associated uveitis Pathway 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 Disease-modifying antirheumatic drugs, 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 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 vitreous opacities, or macular edema) potentially causing further worsening of vision in the short term. E Nonsteroidal anti-inflammatory drugs: As per 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 Cycloplegics: administer cycloplegics to prevent or treat posterior synechiae in patients with active uveitis. E 4. Therapeutic procedures Ocular corticosteroid injections: consider administering ocular corticosteroid injections as rescue therapy in individual patients with unilateral or bilateral severe active uveitis with https://web.pathway.md/diseases/recNlaJhQNTnqs8AH 3/5 6/29/23, 4:07 AM Juvenile idiopathic arthritis-associated uveitis Pathway 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 5. Patient education General counseling: 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 6. Follow-up and surveillance Assessment of treatment response: As per ACR 2019 guidelines, obtain ophthalmic monitoring within 1 month after each change of topical corticosteroids in patients with 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 Treatment tapering: As per 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 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 Clinical findings Symptoms Past medical history Blurred vision JIA Eye pain Headache Photophobia Vision loss Ocular exam Anterior chamber aqueous flare Anterior chamber inflammatory cells https://web.pathway.md/diseases/recNlaJhQNTnqs8AH 4/5 6/29/23, 4:07 AM Juvenile idiopathic arthritis-associated uveitis Pathway Anterior uveitis Macular edema Vitreous opacities intraocular pressure visual acuity References 1. A Heiligenhaus, K Minden, C Tappeiner et al. Update of the evidence based, interdisciplinary guideline for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis. Semin Arthritis Rheum. 2019 Aug;49 1 43 55. Open 2. Tamas Constantin, Ivan Foeldvari, Jordi Anton et al. Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative. Ann Rheum Dis. 2018 Aug;77 8 1107 1117. Open 3. Angeles-Han ST, Ringold S, Beukelman T et al. 2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis- Associated Uveitis. Arthritis Care Res Hoboken). 2019 Jun;71 6 703 716. Open https://web.pathway.md/diseases/recNlaJhQNTnqs8AH 5/5