Differences in Disease Phenotype, Management and Outcomes of Children with Juvenile Idiopathic Arthritis throughout the World – Analysis of 8,325 Patients Enrolled in the Epoca Study

Alessandro Consolaro^1,2, Nicolino Ruperto³, Pieter van Dijkhuizen⁴, Marco Garrone⁵, Mariangela Rinaldi⁵, Jaime De Inocencio⁶, Erkan Demirkaya⁷, Stella Maris Garay⁸, Dirk Föll⁹, Daniel J Lovell¹⁰, Calin Lazar¹¹, Susan Nielsen¹², Berit Flatø¹³, Alberto Martini^1,2, Angelo Ravelli^1,2 and Pediatric Rheumatology International Trials Organization and EPOCA Study Group, ¹Istituto Giannina Gaslini, Genoa, Italy, ²University of Genova, Genova, Italy, ³Paediatric Rheumatology International Trials Organization (PRINTO), Istituto Giannina Gaslini, Genoa, Italy, ⁴Istituto Giannina Gaslini, Genova, Italy, ⁵PRINTO - Istituto Giannina Gaslini, Genova, Italy, ⁶Hospital 12 de Octubre, Madrid, Spain, ⁷Gulhane Military Medical Faculty, Ankara, Turkey, ⁸Hospital de Ninos, La Plata, Argentina, ⁹University Hospital Muenster, Muenster, Germany, ¹⁰PRCSG Cincinnati Children's Hospital Medical Center, Cinncinnati, OH, ¹¹Clinica Pediatrie I, Cluj-Napoca, Romania, ¹²Rigshospitalet, Copenhagen, Denmark, ¹³Department of Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Epidemiologic methods, outcomes and pediatric rheumatology

Session Information

Date: Wednesday, November 16, 2016

Title: ACR/ARHP Combined Abstract Session: Pediatric Rheumatology

Session Type: ACR Concurrent Abstract Session

Session Time: 9:00AM-10:30AM

Background/Purpose: Several epidemiologic surveys have documented a remarkable, yet unexplained, disparity in the prevalence of juvenile idiopathic arthritis (JIA) subtypes among different geographic areas or ethnic groups. Moreover, the therapeutic approach to JIA is not standardized and the availability of the novel and costly biologic medications is not uniform throughout the world. This disparity may have significant impact on disease outcome. The multinational study of the EPidemiology, treatment and Outcome of Childhood Arthritis (EPOCA study) is aimed to obtain information on the variability of JIA phenotypes in different geographic areas, the therapeutic approaches of pediatric rheumatologists practicing in diverse countries, and the disease status and outcome of children with JIA currently followed worldwide.

Methods: Participation in the study was proposed to all pediatric rheumatology centers that are part of the Pediatric Rheumatology International Trials Organization (PRINTO), and to several centers in the US and Canada. Each center was asked to enroll 100 consecutive JIA patients or all consecutive patients seen within 6 months. Each patient received a retrospective and cross-sectional assessment. Parent- and child-reported outcomes were recorded through the administration of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR). Participating countries were grouped into 6 geographic areas. Patients were then grouped according to their country’s gross domestic product per capita (GDP) and the total expenditure on health per capita (HE) (source www.who.org).

Results: Currently, 8,325 patients from 44 countries have been entered in the web database. Comparison of main epidemiology, treatment, and outcome features across the different geographic areas is presented in the table. Patients living in countries with GDP or HE below the median had lower frequency of remission, higher median cJADAS, higher frequency of damage, and were less frequently prescribed biologic DMARDs. These results were confirmed when analyses were conducted by disease category.

	Africa N = 261	Asia N = 874	Eastern Europe N = 2587	Latin America N = 814	North America N = 422	Western Europe N = 3367
Median (IQR) age at onset, years	7 (3.6-10.5)	5.7 (2.9-9.2)	6.4 (2.9-10.5)	6.6 (3.6-10.3)	8.1 (3.7-11.1)	4.1 (2-8.7)
Systemic arthritis	17.2	26.5	8.1	17.7	4.7	6.9
Oligoarthritis	23	31.9	44.1	31.7	33.9	49.2
Uveitis	4.6	5.5	10	6.6	10.4	16.9
Use of biologics	22.6	23.6	28.9	34.2	47.6	39
Median (IQR) cJADAS10	7 (2-11)	2.5 (0-7.8)	4.5 (1-9.5)	2.5 (0-9.5)	2.3 (0-7)	2 (0-6)
Median (IQR) JAFS score	4 (0-8)	1 (0-5)	1 (0-4)	1 (0-5)	1 (0-4)	0 (0-3)
JADI-Articular > 0	27.6	19.2	23.7	31.9	15.2	12.2
JADI-Extraarticular > 0	24.5	16.8	14.8	14.5	5.9	9.1

Data are percentages unless otherwise indicated. cJADAS: clinical (3-item) JADAS; JAFS: Juvenile Arthritis Functionality Scale; JADI: Juvenile Arthritis Damage Index

Conclusion: These results provide further evidence of the wide difference of JIA characteristics across geographic areas in terms of age at disease onset, subtype prevalence, and frequency of anterior uveitis. Overall, patients living in countries with lower GDP or HE had higher levels of disease activity and cumulative damage than patients living in wealthier countries. This disparity may be partially related to differences in the availability or affordability of biologics.

Disclosure: A. Consolaro, None; N. Ruperto, Abbott, BMS, "Francesco Angelini", GlaxoSmithKline (GSK), Hoffman-La Roche, Italfarmaco, Janssen, Novartis, Pfizer, Sanofi Aventis, Schwarz Biosciences, Sobi, Xoma and Wyeth, 2,Abbott, AbbVie, Amgen, Biogenidec, Astellas, Alter, AstraZeneca, Boehringer, BMS, CDPharma, Celgene, CrescendoBio, EMD Serono,Hoffman-La Roche, Italfarmaco, Janssen, MedImmune, Medac, Novartis, Novo Nordisk, Pfizer, Sanofi Aventis, Servier, Takeda and Ver, 8; P. van Dijkhuizen, None; M. Garrone, None; M. Rinaldi, None; J. De Inocencio, None; E. Demirkaya, None; S. M. Garay, None; D. Föll, None; D. J. Lovell, National Institutes of Health, 2,Astra-Zeneca, Bristol Meyers Squibb, AbbVee, Pfizer, Roche, Novartis, UCB, Forest Research Institute, Horizon, Johnson & Johnson, Biogen, Takeda, Genentech, Glaxo Smith Kline, Boehringer Ingelheim, Celgene and Jannsen, 5; C. Lazar, None; S. Nielsen, None; B. Flatø, None; A. Martini, None; A. Ravelli, AbbVie, BMS, Pfizer, Hoffman LaRoche, Novartis, Centocor, 8.

To cite this abstract in AMA style:

Consolaro A, Ruperto N, van Dijkhuizen P, Garrone M, Rinaldi M, De Inocencio J, Demirkaya E, Garay SM, Föll D, Lovell DJ, Lazar C, Nielsen S, Flatø B, Martini A, Ravelli A. Differences in Disease Phenotype, Management and Outcomes of Children with Juvenile Idiopathic Arthritis throughout the World – Analysis of 8,325 Patients Enrolled in the Epoca Study [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/differences-in-disease-phenotype-management-and-outcomes-of-children-with-juvenile-idiopathic-arthritis-throughout-the-world-analysis-of-8325-patients-enrolled-in-the-epoca-study/. Accessed .

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