Consensus-Based Recommendations for the Management of Uveitis Associated with Juvenile Idiopathic Arthritis
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ARD Online First, published on March 28, 2018 as 10.1136/annrheumdis-2018-213131 Clinical and epidemiological research Ann Rheum Dis: first published as 10.1136/annrheumdis-2018-213131 on 28 March 2018. Downloaded from EXTENDED REPORT Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative Tamas Constantin,1 Ivan Foeldvari,2 Jordi Anton,3 Joke de Boer,4 Severine Czitrom -Guillaume,5 Clive Edelsten,6 Raz Gepstein,7 Arnd Heiligenhaus,8,9 Clarissa A Pilkington,10 Gabriele Simonini,11 Yosef Uziel,12 Sebastian J Vastert,13 Nico M Wulffraat,13 Anne-Mieke Haasnoot,4 Karoline Walscheid,8 Annamária Pálinkás,1 Reshma Pattani,6 Zoltán Györgyi,1 Richárd Kozma,1 Victor Boom,14 Andrea Ponyi,1 Angelo Ravelli,15 Athimalaipet V Ramanan16 Handling editor Josef S ABSTRacT adolescents with rheumatic diseases. The European Smolen Background In 2012, a European initiative called League against Rheumatism (EULAR) has produced Single Hub and Access point for pediatric Rheumatology a number of recommendations in the area of paedi- For numbered affiliations see 1 end of article. in Europe (SHARE) was launched to optimise and atric rheumatology such as juvenile dermatomyositis disseminate diagnostic and management regimens in using a standardised procedure2 and referring to a Correspondence to Europe for children and young adults with rheumatic generalised instrument for guideline assessment: the Prof Athimalaipet V Ramanan, diseases. Juvenile idiopathic arthritis (JIA) is the Appraisal of Guidelines for Research & Evaluation.3 University Hospitals Bristol most common rheumatic disease in children and Juvenile idiopathic arthritis (JIA) is the most NHS Foundation Trust & Bristol Medical School, University of uveitis is possibly its most devastating extra-articular common rheumatic disease in children, and uveitis Bristol, Bristol BS8 1TH, UK; manifestation. Evidence-based guidelines are sparse and is the most frequent and potentially most devastating avramanan@ hotmail. com management is mostly based on physicians’ experience. extra-articular manifestation. There are no interna- Consequently, treatment practices differ widely, within tional consensus statements specifically relating to Received 28 January 2018 and between nations. the diagnosis and treatment of JIA-associated uveitis Revised 7 March 2018 Accepted 11 March 2018 Objectives To provide recommendations for the although there are national guidelines from Germany diagnosis and treatment of JIA-associated uveitis. and Spain.4 5 Management is therefore based on physi- Methods Recommendations were developed by cians’ personal experience; and considerable variation an evidence-informed consensus process using the in clinical practice, both in terms of investigation and European League Against Rheumatism standard management of JIA-associated uveitis, was found in an operating procedures. A committee was constituted, extensive survey of uveitis experts.6 consisting of nine experienced paediatric rheumatologists With the rapid development of novel therapies and three experts in ophthalmology from Europe. for JIA, clear recommendations based on avail- http://ard.bmj.com/ Recommendations derived from a validated systematic able best evidence and expert opinion (when trial literature review were evaluated by an Expert Committee evidence is lacking) will help physicians in the care and subsequently discussed at two consensus meetings of patients with JIA-associated uveitis. The uveitis using nominal group techniques. Recommendations were seen with JIA is usually chronic anterior uveitis accepted if >80% agreement was reached (including all which is asymptomatic, but acute anterior uveitis three ophthalmologists). can also be seen in the enthesitis-related arthritis Results In total, 22 recommendations were accepted subtype.7 8 There is a clear need to regularly update on October 2, 2021 by guest. Protected copyright. (with >80% agreement among experts): 3 on diagnosis, those managing patients with JIA uveitis through 5 on disease activity measurements, 12 on treatment and expert opinion. While the majority of treatments 2 on future recommendations. available for the treatment of arthritis have a solid Conclusions The SHARE initiative aims to identify best evidence base from clinical trials, corresponding practices for treatment of patients suffering from JIA- data for patients with uveitis may not be known or associated uveitis. Within this remit, recommendations the level of evidence may not be as strong at the for the diagnosis and treatment of JIA-associated time of their introduction into clinical practice. uveitis have been formulated by an evidence-informed The primary aims of the recommendations consensus process to suggest a standard of care for JIA- forwarded by the Expert Group were to develop associated uveitis patients throughout Europe. agreed strategies to ► prevent or reduce the likelihood of JIA-associ- ated uveitis from occurring and minimise the damage at the time of diagnosis To cite: Constantin T, ► recommend treatments and management Foeldvari I, Anton J, et al. INTRODUCTION Ann Rheum Dis Epub ahead In 2012, Single Hub and Access point for pediatric strategies that would reduce inflammation of print: [please include Day Rheumatology in Europe (SHARE) was launched and prevent the development of those ocular Month Year]. doi:10.1136/ with the aim of optimising and disseminating diag- complications most likely to cause irreversible annrheumdis-2018-213131 nostic and management regimens for children and visual loss. Constantin T, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213131 1 Copyright Article author (or their employer) 2018. Produced by BMJ Publishing Group Ltd (& EULAR) under licence. Clinical and epidemiological research Ann Rheum Dis: first published as 10.1136/annrheumdis-2018-213131 on 28 March 2018. Downloaded from METHODS searched in Medical Subject Headings/Emtree terms, titles A committee of 12 experts (AH, BV, CP, CE, SC-G, IF, JdB, and abstracts (figure 1). Reference tracking was performed in JA, KW, RG, YU, NW) in paediatric rheumatology (n=9) or all included studies (full search strategy is shown in figure 1). ophthalmology (n=3) was established to develop recommenda- Experts (TC, AP, VB) selected papers relevant to uveitis asso- tions for JIA-associated uveitis based on consensus, but evidence ciated with JIA investigations and/or treatment to be taken informed, using EULAR standard operating procedures for forward for validity assessment (inclusion and exclusion criteria developing best practice.2 3 shown in figure 1) by members of the expert committee. Systematic literature search Validity assessment The electronic databases PubMed/MEDLINE, Embase and The selected articles were randomly allocated to the expert Cochrane were searched independently by two researchers for group, and two members per paper independently assessed the eligible articles in February 2015. All synonyms of JIA were methodological quality of those papers meeting the inclusion http://ard.bmj.com/ on October 2, 2021 by guest. Protected copyright. Figure 1 Summary of search strategy for identification of key articles. 2 Constantin T, et al. Ann Rheum Dis 2018;0:1–11. doi:10.1136/annrheumdis-2018-213131 Clinical and epidemiological research Ann Rheum Dis: first published as 10.1136/annrheumdis-2018-213131 on 28 March 2018. Downloaded from Table 1 Recommendations for diagnosis and screening in juvenile idiopathic arthritis (JIA)-related uveitis Recommendation L S Agreement (%) References 1. All patients in whom a diagnosis of JIA is being considered should be screened for uveitis according to a 2A B 100 13–32 contemporary and audited protocol. Formal screening protocol should be administered in all centres, where patients with JIA are seen. 2. Frequency of ophthalmological follow-up visits must be based on disease severity and needs to be decided in 4 D 100 13–27 33–66 conjunction with an expert ophthalmologist. 3. Patients with JIA stopping any systemic immunosuppressant are at risk of developing new onset uveitis or 2B B 100 67–70 recurrence of uveitis after a prolonged remission. After stopping systemic immunosuppression, it is recommended that all patients with JIA are screened by an ophthalmologist at least every three months for at least 1 year. Agreement indicates the % of experts that agreed on the recommendation during the final voting round of the consensus meeting. 1A, meta-analysis of cohort studies; 1B, meta-analysis of case–control studies; 2A, cohort studies; 2B, case–control studies; 3, non-comparative descriptive studies; 4, expert opinion; A, based on level 1 evidence; B, based on level 2 or extrapolated from level 1; C, based on level 3 or extrapolated from level 1 or 2; D, based on level 4 or extrapolated from level 3 or 4 expert opinion. L, level of evidence; S, strength of evidence. criteria (figure 1). Data were extracted using predefined scoring these, 117 were selected to support the development of consen- forms for diagnostic and therapeutic studies. Disagreements sus-based recommendations by the expert group (figure 1). were resolved by discussion or by the opinion of a third expert. Adapted classification tables for diagnostic, therapeutic and Recommendations epidemiological studies were used to determine the level of The sections that follow report the recommendations of the expert 9–11 evidence and strength of each recommendation. committee based on the supporting literature.13–128