Jessica TibaldiAngela PistorioElena AlderaLaura PuzoneYasser El MiedanyPriyankar PalPrabhas Prasun GiriHriday DeRaju KhubchandaniPallavi Pimpale ChavanSoamarat VilaiyukButsabong LerkvaleekulJutamas YamsuwanTapas K. SabuiPragati DattaManuela PardeoClaudia BracagliaSujata SawhneySumidha MittalWaleed A. HassanGhada Farouk ElderinyMohammed Hassan Abu-ZaidMervat EissaFlavio SztajnbokFernanda C. das Neves SztajnbokRicardo RussoMaría Martha KatsicasRolando CimazEdoardo MarraniEkaterina AlexeevaTatyana M. DvoryakovskayaMotasem O. AlsuweitiRa'ed M. AlzyoudMikhail KostikIrina ChikovaFrancesca MinoiaGiovanni FilocamoYomna FaragHala LotfySamah Ismail NasefSulaiman M. Al-MayoufMaria Cristina MaggioClaudia Saad MagalhaesRomina GallizziGiovanni ContiMasaki ShimizuAdele CivinoEnrico FeliciGabriella GiancaneNicolino RupertoAlessandro ConsolaroAngelo RavelliOspedale Vito FazziAzienda Ospedaliero Universitaria MeyerAzienda Ospedaliera Nazionale Santi Antonio e Biagio e Cesare ArrigoAlexandria UniversityUniversità degli Studi di MilanoBenha UniversityUniversità degli Studi di GenovaKanazawa UniversitySir Ganga Ram HospitalSuez Canal UniversitySaint Petersburg State Pediatric Medical UniversityIRCCS Ospedale Pediatrico Bambino GesùUniversità degli Studi di PalermoKing Faisal Specialist Hospital and Research CentreIRCCS Istituto Giannina Gaslini - Ospedale PediatricoFaculty of Medicine, Ramathibodi Hospital, Mahidol UniversityAin Shams University, Faculty of MedicineUniversità degli Studi di Messina, Facoltà di Medicina e ChirurgiaUniversity of TantaCairo UniversitySechenov First Moscow State Medical UniversityOspedale Maggiore Policlinico MilanoUNESP-Universidade Estadual PaulistaUniversidade do Estado do Rio de JaneiroMedical College and Hospital KolkataFundacion Hospital de Pediatria Professor Dr. Juan P. GarrahanUniversidade Federal do Rio de JaneiroSRCC Children's HospitalQueen Rania Children HospitalInstitute of Child HealthNational Medical Research Center of Children’s Health2020-11-182020-11-182020-11-01Rheumatology (Oxford, England). Vol.59, No.11 (2020), 3505-3514146203322-s2.0-85094932332https://repository.li.mahidol.ac.th/handle/20.500.14594/60037© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com. OBJECTIVE: To develop a composite disease activity score for systemic JIA (sJIA) and to provide preliminary evidence of its validity. METHODS: The systemic Juvenile Arthritis Disease Activity Score (sJADAS) was constructed by adding to the four items of the original JADAS a fifth item that aimed to quantify the activity of systemic features. Validation analyses were conducted on patients with definite or probable/possible sJIA enrolled at first visit or at the time of a flare, who had active systemic manifestations, which should include fever. Patients were reassessed 2 weeks to 3 months after baseline. Three versions were examined, including ESR, CRP or no acute-phase reactant. RESULTS: A total of 163 patients were included at 30 centres in 10 countries. The sJADAS was found to be feasible and to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha 0.64-0.65), fair ability to discriminate between patients with different disease activity states and between those whose parents were satisfied or not satisfied with illness outcome (P < 0.0001 for both), and strong responsiveness to change over time (standardized response mean 2.04-2.58). Overall, these properties were found to be better than those of the original JADAS and of DAS for RA and of Puchot score for adult-onset Still's disease. CONCLUSION: The sJADAS showed good measurement properties and is therefore a valid instrument for the assessment of disease activity in children with sJIA. The performance of the new tool should be further examined in other patient cohorts that are evaluated prospectively.Mahidol UniversityMedicineArticleSCOPUS10.1093/rheumatology/keaa240