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Abstract Number: 092

The Initial Treatment of Systemic Juvenile Idiopathic Arthritis: An International Collaboration Among 10 Registries

Mary Beth Son1, Yukiko Kimura 2, Kristiina Aalto 3, Lillemor Berntson 4, Johnathan Dallas 1, Ciaran Duffy 5, Mia Glerup 6, Jaime Guzman 7, Troels Herlin 8, Petteri Hovi 9, Kimme Hyrich 10, Jens Klotsche 11, Bo Magnusson 12, Vanessa McIntyre 13, Ellen Nordal 14, Seza Ozen 15, Maria Jose Santos 16, Betul Sozeri 17 and Timothy Beukelman 18, 1Boston Children's Hospital, Boston, Massachusetts, 2Hackensack Meridian School of Medicine, Hackensack, 3Department of Pediatrics, New Children's Hospital, Helsinki University Hospital, Pediatric Research Center, University of Helsinki, Helsinki, Finland., HUS, Finland, 4Department of Womens and Childrens Health, Uppsala University, Uppsala, Sweden., Uppsala, Sweden, 5Ottawa, Canada, 6Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, Aarhus, Denmark, 7University of British Columbia and BC Children's Hospital, Vancouver, Canada, 8Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark., Aarhus N, Denmark, 9Helsinki University Hospital, Helsinki, Finland, 10Manchester, United Kingdom, 11Berlin, Germany, 12Karolinska University Hospital, Stockholm, Sweden, 13Manchester University, Manchester UK, United Kingdom, 14Department of Pediatrics, University Hospital of North Norway, and Department of Clinical Medicine, UiT The Arctic University of Norway, Tromsø, Norway., Tromsø, Norway, 15Hacettepe University, Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, 16Portugal, 17Boston, Turkey, 18University of Alabama at Birmingham, Birmingham

Meeting: 2020 Pediatric Rheumatology Symposium

Keywords: Biologic agents, corticosteroids, medication, registries, Still's disease

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Session Information

The 2020 Pediatric Rheumatology Symposium, originally scheduled for April 29 – May 2, was postponed due to COVID-19; therefore, abstracts were not presented as scheduled.

Date: Friday, May 1, 2020

Session Title: Poster Session 2

Session Type: ACR Abstract Session

Session Time: 5:00PM-6:00PM

Background/Purpose: The introduction of biologic medications has revolutionized the care of children with systemic juvenile idiopathic arthritis (SJIA). Differences in treatment approaches among different countries and how they have changed in the modern era of biologic medications have not been well studied. We sought to contrast the presenting features, initial treatment and 12-month outcomes of SJIA patients in pediatric rheumatology Registries in the United States, Canada, United Kingdom, Portugal, Sweden, Denmark, Turkey, Germany, Norway and Finland using individual country registry data.

Methods: Data were extracted locally from the 10 JIA Registries. Disease manifestations collected within nine weeks of first visit with a pediatric rheumatologist (PR) or at diagnosis were included as presenting features. Data on medication use within first year of diagnosis were collected. Clinical outcomes, including the Physician Global Assessment (PGA), active joint count (AJC) and current glucocorticoid (GC) use, were collected at the 12±3 month visit following diagnosis. To assess modern era treatment approaches, we compared data for children treated before and after 2012. Weighted means were used to adjust for varying numbers of patients per Registry.

Results: A total of 1,149 patients were included for demographic data; 553 patients had medication data for the 2012-2018 era. The cohort was predominantly female and Caucasian; median age at first presentation to PR ranged from 5.3-8 years. Median duration of symptoms prior to first visit with PR varied from 0-3.3 months. GC use was common in the first year of diagnosis, with a weighted average of 72% (range 33-96%) of patients prescribed GC in the first year over the duration of each Registry’s collection. Biologic use included IL-1, IL-6 and TNF inhibitors. There was a clear increase in the proportion of patients treated with biologics, primarily anakinra, after 2012 (Table 1). Weighted mean PGA and AJC at the 12±3 month visit were 1.55 and 1.57, respectively (Table 2).  At the 12±3 month visit, the proportion of patients prescribed GC varied (weighted mean 40%, range 26-60%).

Conclusion: Analysis of SJIA patients across 10 international Registries demonstrated that patients were seen by a PR at variable times from symptom onset. Although many local factors may influence treatment decisions, biologic use increased after 2011, consistent with secular trends. Anakinra was the most frequently prescribed biologic. Nearly 75% of patients were prescribed steroids within the first year of diagnosis; steroid use seemed to decrease at 12 months of treatment. More study is needed to determine long-term outcomes in SJIA patients treated with modern treatment regimens.


Disclosure: M. Son, None; Y. Kimura, Genentech, 1, CARRA, 1; K. Aalto, None; L. Berntson, None; J. Dallas, None; C. Duffy, None; M. Glerup, None; J. Guzman, None; T. Herlin, None; P. Hovi, None; K. Hyrich, Abbvie, 1, UCB, 1, Bristol Myers Squibb, 1, Pfizer, 1; J. Klotsche, None; B. Magnusson, None; V. McIntyre, None; E. Nordal, None; S. Ozen, None; M. Santos, None; B. Sozeri, None; T. Beukelman, UCB, 1, Novartis, 1.

To cite this abstract in AMA style:

Son M, Kimura Y, Aalto K, Berntson L, Dallas J, Duffy C, Glerup M, Guzman J, Herlin T, Hovi P, Hyrich K, Klotsche J, Magnusson B, McIntyre V, Nordal E, Ozen S, Santos M, Sozeri B, Beukelman T. The Initial Treatment of Systemic Juvenile Idiopathic Arthritis: An International Collaboration Among 10 Registries [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 4). https://acrabstracts.org/abstract/the-initial-treatment-of-systemic-juvenile-idiopathic-arthritis-an-international-collaboration-among-10-registries/. Accessed January 21, 2021.
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