Jessica Tibaldi1,2, Angela Pistorio3, Elena Aldera2, Laura Puzone2, Yasser El Miedany4, Priyankar Pal5, Prabhas Prasun Giri5, Hriday De5, Raju Khubchandani6, Pallavi Pimpale Chavan6, Soamarat Vilaiyuk7, Butsabong Lerkvaleekul7, Jutamas Yamsuwan7, Tapas K Sabui8, Pragati Datta8, Manuela Pardeo9, Claudia Bracaglia9, Sujata Sawhney10, Sumidha Mittal10, Waleed A Hassan11, Ghada Farouk Elderiny12, Mohammed Hassan Abu-Zaid13, Mervat Eissa14, Flavio Sztajnbok15, Fernanda C das Neves Sztajnbok16, Ricardo Russo17, María Martha Katsicas17, Rolando Cimaz18, Edoardo Marrani19, Ekaterina Alexeeva20,21, Tatyana M Dvoryakovskaya20,21, Motasem O Alsuweiti22, Ra'ed M Alzyoud22, Mikhail Kostik23, Irina Chikova23, Francesca Minoia24, Giovanni Filocamo24, Yomna Farag14, Hala Lotfy14, Samah Ismail Nasef25, Sulaiman M Al-Mayouf26, Maria Cristina Maggio27, Claudia Saad Magalhaes28, Romina Gallizzi29, Giovanni Conti30, Masaki Shimizu31, Adele Civino32, Enrico Felici33, Gabriella Giancane1,2, Nicolino Ruperto1, Alessandro Consolaro1,2, Angelo Ravelli1,2,21. 1. UOC Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 2. Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DiNOGMI), Università degli Studi di Genova, Genoa, Italy. 3. Dipartimento di Epidemiologia e Biostatistica, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 4. Faculty of Medicine, Ain Shams University, Cairo, Egypt. 5. Pediatric Rheumatology Division, Institute of Child Health, Kolkata, India. 6. Section of Pediatric Rheumatology, SRCC Children's Hospital, Mumbai, India. 7. Rheumatology Division, Pediatric Department, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. 8. Pediatric Rheumatology Clinic, R G Kar Medical College, Kolkata, India. 9. Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy. 10. Division of Pediatric Rheumatology, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi, India. 11. Faculty of Medicine, Benha University, Benha, Egypt. 12. Faculty of Medicine, Alexandria University, Alexandria, Egypt. 13. Faculty of Medicine, Tanta University, Tanta, Egypt. 14. Faculty of Medicine, Cairo University, Cairo, Egypt. 15. Pediatric Rheumatology Division, Adolescent Health Care Unit, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil. 16. Department of Internal Medicine, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. 17. Servicio de Inmunología y Reumatología, Hospital de Pediatría Garrahan, Buenos Aires, Argentina. 18. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. 19. Division of Rheumatology, University Hospital Meyer, Florence, Italy. 20. Rheumatology Division, National Medical Research Center of Children's Health, Moscow, Russian Federation. 21. Sechenov First Moscow State Medical University, Moscow, Russian Federation. 22. Department of Immunology, Rheumatology and Allergy, Queen Rania Children's Hospital, Amman, Jordan. 23. Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russian Federation. 24. UOC Pediatria a Media Intensità di Cure, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. 25. Rheumatology Department, Faculty of Medicine, Suez Canal University, Ismailia, Egypt. 26. Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. 27. Dipartimento Promise G. D'Alessandro, Università degli Studi di Palermo, Palermo, Italy. 28. Pediatric Department, Hospital das Clínicas - Botucatu Medicine University, UNESP, Botucatu, Brazil. 29. UOC Pediatria, Servizio di Immuno-Reumatologia Pediatrica, Azienda Ospedaliera Universitaria Gaetano Martino Messina, Messina, Italy. 30. UO Nefrologia e Reumatologia Pediatrica, Azienda Ospedaliera Universitaria Gaetano Martino, Messina, Italy. 31. Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan. 32. Pediatric Unit, Ospedale Vito Fazzi, Lecce, Italy. 33. Pediatric Unit, AON SS Antonio e Biagio e Cesare Arrigo Children's Hospital, Alessandria, Italy.
Abstract
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.
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.
Authors: Alberto Martini; Daniel J Lovell; Salvatore Albani; Hermine I Brunner; Kimme L Hyrich; Susan D Thompson; Nicolino Ruperto Journal: Nat Rev Dis Primers Date: 2022-01-27 Impact factor: 65.038
Authors: Reza Yarani; Ali Shojaeian; Oana Palasca; Nadezhda T Doncheva; Lars Juhl Jensen; Jan Gorodkin; Flemming Pociot Journal: Front Immunol Date: 2022-06-06 Impact factor: 8.786