The SHARE Recommendations on Diagnosis and Treatment of Systemic JIA

Arjen Leek¹, Jordi Anton², Tadej Avcin³, Fabrizio De Benedetti⁴, Victor Boom¹, Claudia Bracaglia⁵, Paul Brogan⁶, Tamas Constantin⁷, Alessandro Consolaro⁸, Pavla Dolezalova⁹, Despina Eleftheriou¹⁰, Helen Foster¹¹, Claas Hinze¹², Isabelle Koné-Paut¹³, Kirsten Minden¹⁴, Francesca Minoia¹⁵, Pierre Quartier¹⁶, Angelo Ravelli⁸, Nicolino Ruperto¹⁷, Joost Swart¹⁸, Yosef Uziel¹⁹, Helmut Wittkowski²⁰, Carine Wouters²¹, Mojca Zajc Avramovitz²², Nico Wulffraat¹⁸ and Sebastiaan Vastert¹, ¹University Medical Center Utrecht, Utrecht, Netherlands, ²Sant Joan de Déu Hospital, Madrid, Spain, ³Ljubljana University Medical Centre, Ljubljana, Slovenia, ⁴Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁵Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁶UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁷Semmelweiss University Hospital, Budapest, Hungary, ⁸Università degli Studi di Genova, Genoa, Italy, ⁹University Hospital Prague, Prague, Czech Republic, ¹⁰UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Section Head Infection, Immunology, and Rheumatology, London, United Kingdom, ¹¹Newcastle University, Newcastle upon Tyne, United Kingdom, ¹²University Hospital M�nster, M�nster, Germany, ¹³Necker Hospital, Paris, France, ¹⁴Deutsches Rheuma-Forschungszentrum Berlin, Berlin, Germany, ¹⁵Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, Milan, Italy, ¹⁶Necker-Enfants Malades University Hospital, Assistance Publique-Hopitaux de Paris, Paris, France, ¹⁷Istituto Giannina Gaslini, Genova, Italy, ¹⁸IRCCS Istituto Giannina Gaslini, PRINTO, Genoa, Italy, Genova, Italy, ¹⁹Meir Medical Center, Kfar Saba, Israel, ²⁰University Hospital Muenster, Muenster, Germany, ²¹University Hospital Leuven, Leuven, Belgium, ²²University Hospital Ljubljana, Ljubljana, Slovenia

Meeting: ACR Convergence 2020

Keywords: Clinical practice guidelines, Juvenile idiopathic arthritis, Still's disease

Session Information

Date: Sunday, November 8, 2020

Title: Pediatric Rheumatology – Clinical Poster II: Systemic JIA, Autoinflammatory, & Scleroderma

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a rare, complex auto-inflammatory disease with significant morbidity including fever, rash, serositis and articular problems. With the availability of interleukin-1 (IL-1) and IL-6 inhibitor treatment, morbidity has significantly reduced and the (long term) outcome for sJIA patients has improved over the last decade. However, differences in access to care and differences in treatment strategies between countries in and outside of Europe remain a concern.

The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) consortium aimed to develop best practices for paediatric rheumatic diseases in order to decrease differences in care between European countries. Here, we present the final results of the literature review and a series of consensus meetings on defining overarching, diagnostic and therapeutic recommendations for diagnosis and treatment of systemic JIA.

Methods: The SHARE methodology concerning consensus-based principles and guidelines has been previously published, including the use of the EULAR standardized operating procedure for developing best practice recommendations(1). As per these guidelines, a methodologist provided supervision during the process and consensus meetings.

A systematic literature search of the Medline, Embase and Cochrane databases was performed in June 2013 and this was updated in November 2019. Results were used to develop and support recommendations on diagnosis, treatment and complications of systemic JIA. These recommendations were pre-tested in an online survey to a task-force of expert paediatric rheumatologists to assess potential agreement and enable rewording. The participating experts convened in 3 consensus meetings (Genua 2014, Barcelona 2015 and Utrecht 2020) to develop the best practices. Making use of the Nominal Group Technique, recommendations were proposed, discussed and voted on. Recommendations that reached ≥80% were accepted in this guideline.

Results: The 2-step systematic literature review finally included 98 papers on sJIA. Quality and grade of evidence was assessed and a categorized overview was used as backbone and reference during the consensus meeting. In total, 15 recommendations were developed and accepted in the final consensus meeting: 4 overarching principles, 3 diagnostic recommendations and 8 recommendations on treatment of sJIA and its complications. Recommendations are presented with accompanying level of evidence (LoE), strength of recommendation (SoR) and percentage of agreement (PoA). In addition, adherence to the 2018 EULAR Treat-to-Target principles on JIA was confirmed by the expert panel.

Conclusion: These SHARE best practices on sJIA are based on the best available evidence and expert opinion, and provide recommendations for the diagnosis and treatment of patients with sJIA, aiming to improve the outcome for all sJIA patients in Europe and beyond.

Disclosure: A. Leek, None; J. Anton, Sobi, 1, 2, 3, Novartis, 1, 2, 3, GSK, 1, 2, 3, Pfizer, 1, 2, BMS, 1, Chemocentryx,, 1, Lilly, 1, Novimmune, 1, Sanofi, 1, Roche, 1; T. Avcin, AbbVie, 5, 8, Alexion, 5, Octapharma, 5, 8, Takeda, 5, 8; F. De Benedetti, Novartis, 2, 8, Novimmune, 2, 9, Sobi, 2, 8, 9, Roche, 2, 8, Pfizer, 2, Sanofi, 2, AbbVie, 8; V. Boom, None; C. Bracaglia, SOBI, 8; P. Brogan, Sobi, Roche, Novartis, 2, 5; T. Constantin, None; A. Consolaro, None; P. Dolezalova, SOBI, 5; D. Eleftheriou, None; H. Foster, Sanofi-Genzyme, 2; C. Hinze, Novartis, 5; I. Koné-Paut, None; K. Minden, Sanofi, 1, gsk, 1, Roche, 1, Abbvie, 1, Biermann, 8, Medac, 8; F. Minoia, None; P. Quartier, AbbVie, 1, Bristol-Myers Squibb, 1, Chugai-Roche, 1, Lilly, 1, Novartis, 1, Novimmune, 1, Sanofi, 1, Sobi, 1; A. Ravelli, AbbVie, BMS, Pfizer, Hoffman LaRoche, Novartis, Centocor, "Francesco Angelini" and Reckitt Benckiser., 1, 2; N. Ruperto, Ablynx, 5, 8, Astrazeneca-Medimmune, 5, 8, Biogen, 5, 8, Boehringer, 5, 8, Bristol Myers Squibb, 2, 5, 8, 9, Eli Lilly, 2, 5, 8, 9, EMD Serono, 5, 8, GlaxoSmithKline, 2, 5, 8, 9, F Hoffmann-La Roche, 2, 5, 8, 9, Janssen, 2, 5, 8, 9, Merck, 5, 8, Novartis, 2, 5, 8, 9, Pfizer, 2, 5, 8, 9, R-Pharma, 5, 8, Sanofi, 5, 8, Servier, 5, 8, Sinergie, 5, 8, Sobi, 2, 5, 8, 9, AbbVie, 5, 8, Takeda, 5, 8; J. Swart, SOBI, 8; Y. Uziel, Pfizer, 8; H. Wittkowski, Novartis, 8; C. Wouters, Novartis, 2; M. Zajc Avramovitz, None; N. Wulffraat, SOBI, 2; S. Vastert, Sobi, 1, 2, Novartis, 1.

To cite this abstract in AMA style:

Leek A, Anton J, Avcin T, De Benedetti F, Boom V, Bracaglia C, Brogan P, Constantin T, Consolaro A, Dolezalova P, Eleftheriou D, Foster H, Hinze C, Koné-Paut I, Minden K, Minoia F, Quartier P, Ravelli A, Ruperto N, Swart J, Uziel Y, Wittkowski H, Wouters C, Zajc Avramovitz M, Wulffraat N, Vastert S. The SHARE Recommendations on Diagnosis and Treatment of Systemic JIA [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/the-share-recommendations-on-diagnosis-and-treatment-of-systemic-jia/. Accessed .

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