Defining criteria for disease activity states in systemic juvenile idiopathic arthritis based on the systemic Juvenile Arthritis Disease Activity Score

Journal article

Rosina, Silvia, Rebollo‐Gimenez, Ana I., Tarantola, Letizia, Pistorio, Angela, Vyzhga, Y., El Miedany, Yasser, Lotfy, Hala M., Abu‐Shady, Hend, Eissa, M., Osman, Naglaa S., Hassan, Waleed, Mahgoub, Marwa Y., Fouad, Nermeen A., Mosa, D., Adel, Yasmin, Mohamed, Sheren E. M., Radwan, Ahmed R., Abu-Zaid, M., Tabra, Samar A. A., Shalaby, Radwa H., Nasef, S., Khubchandani, Raju, Khan, A., Maldar, Naziya P., Ozen, S., Bayindir, Yagmur, Alsuweiti, Motasem, Alzyoud, Raed, Almaaitah, Hiba, Vilaiyuk, Soamarat, Lerkvaleekul, Butsabong, Alexeeva, Ekaterina, Dvoryakovskaya, Tatyana, Kriulin, Ivan, Bracaglia, C., Pardeo, M., De Benedetti, F., Licciardi, F., Montin, Davide, Robasto, Francesca, Minoia, F., Filocamo, Giovanni, Rossano, Martina, Simonini, Gabriele, Marrani, Edoardo, Abu‐Rumeleih, S., Kostik, M., Belozerov, Konstantin E., Pal, P., Bathia, Jigna N., Katsicas, María M., Villarreal, Giselle, Marino, Achille, Costi, Stefania, Sztajnbok, Flavio, Silva, Rodrigo M., Maggio, Maria C., El‐Ghoneimy, Dalia H., El Owaidy, R., Civino, Adele, Diomeda, Federico, Al‐Mayouf, Sulaiman M., Al‐Sofyani, Fuad, Dāvidsone, Zane, Patrone, Elisa, Saad‐Magalhães, Claudia, Consolaro, A. and Ravelli, A. 2024. Defining criteria for disease activity states in systemic juvenile idiopathic arthritis based on the systemic Juvenile Arthritis Disease Activity Score. Arthritis & Rheumatology. https://doi.org/10.1002/art.42865
AuthorsRosina, Silvia, Rebollo‐Gimenez, Ana I., Tarantola, Letizia, Pistorio, Angela, Vyzhga, Y., El Miedany, Yasser, Lotfy, Hala M., Abu‐Shady, Hend, Eissa, M., Osman, Naglaa S., Hassan, Waleed, Mahgoub, Marwa Y., Fouad, Nermeen A., Mosa, D., Adel, Yasmin, Mohamed, Sheren E. M., Radwan, Ahmed R., Abu-Zaid, M., Tabra, Samar A. A., Shalaby, Radwa H., Nasef, S., Khubchandani, Raju, Khan, A., Maldar, Naziya P., Ozen, S., Bayindir, Yagmur, Alsuweiti, Motasem, Alzyoud, Raed, Almaaitah, Hiba, Vilaiyuk, Soamarat, Lerkvaleekul, Butsabong, Alexeeva, Ekaterina, Dvoryakovskaya, Tatyana, Kriulin, Ivan, Bracaglia, C., Pardeo, M., De Benedetti, F., Licciardi, F., Montin, Davide, Robasto, Francesca, Minoia, F., Filocamo, Giovanni, Rossano, Martina, Simonini, Gabriele, Marrani, Edoardo, Abu‐Rumeleih, S., Kostik, M., Belozerov, Konstantin E., Pal, P., Bathia, Jigna N., Katsicas, María M., Villarreal, Giselle, Marino, Achille, Costi, Stefania, Sztajnbok, Flavio, Silva, Rodrigo M., Maggio, Maria C., El‐Ghoneimy, Dalia H., El Owaidy, R., Civino, Adele, Diomeda, Federico, Al‐Mayouf, Sulaiman M., Al‐Sofyani, Fuad, Dāvidsone, Zane, Patrone, Elisa, Saad‐Magalhães, Claudia, Consolaro, A. and Ravelli, A.
Abstract

Objective
To develop and validate cutoff values in the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) that distinguish the states of inactive disease (ID), minimal disease activity (MiDA), moderate disease activity (MoDA), and high disease activity (HDA) in children with systemic juvenile idiopathic arthritis (sJIA), based on subjective disease state assessment by the treating pediatric rheumatologist.

Methods
The cutoffs definition cohort was composed of 400 patients enrolled at 30 pediatric rheumatology centers in 11 countries. Using the subjective physician rating as an external criterion, 6 methods were applied to identify the cutoffs: mapping, calculation of percentiles of cumulative score distribution, Youden index, 90% specificity, maximum agreement, and ROC curve analysis. Sixty percent of the patients were assigned to the definition cohort and 40% to the validation cohort. Cutoff validation was conducted by assessing discriminative ability.

Results
The sJADAS10 cutoffs that separated ID from MiDA, MiDA from MoDA, and MoDA from HDA were ≤ 2.9, ≤ 10, and > 20.6. The cutoffs discriminated strongly among different levels of pain, between patients with or without morning stiffness, and between patients whose parents judged their disease status as remission or persistent activity/flare or were satisfied or not satisfied with current illness outcome.

Conclusion
The sJADAS cutoffs revealed good metrologic properties in both definition and validation cohorts, and are therefore suitable for use in clinical trials and routine practice.

KeywordsDisease activity states; Systemtic juvenile idiopathic arthritis
Year2024
JournalArthritis & Rheumatology
PublisherWiley
ISSN2326-5191
2326-5205
Digital Object Identifier (DOI)https://doi.org/10.1002/art.42865
Official URLhttps://acrjournals.onlinelibrary.wiley.com/doi/10.1002/art.42865
Publication dates
Online29 Apr 2024
Publication process dates
Accepted03 Apr 2024
Deposited09 May 2024
Accepted author manuscript
License
File Access Level
Open
Output statusPublished
Licensehttp://creativecommons.org/licenses/by-nc-nd/4.0/
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