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Abstract Number: 1925

Discriminant and Predictive Ability of the Parent Version of the Juvenile Arthritis Disease Activity Score in Two Large Multination Cohorts of Patients with Juvenile Idiopathic Arthritis

Francesca Ridella 1, Giedre Januskeviciute 2, Chiara Trincianti 3, Evert Hendrik Pieter Van Dijkhuizen 4, Gabriella Giancane 5, Serena Pastore 6, Kirsten Minden 7, Maria Ekelund 8, Patrizia Barone 9, Matilda Laday 10, Nicolino Ruperto 11, Angelo Ravelli 12 and Alessandro Consolaro13, 1Università degli Studi di Genova, Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DiNOGMI), Genoa, Italy, Genova, Italy, 2Klaipeda Children's Hospital, Klaipeda, Lithuania, Klaipeda, Lithuania, 3Università degli Studi di Genova, Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DiNOGMI), Genoa, Italy, Genoa, Italy, 4Wilhelmina Children’s Hospital, Department of Pediatric Immunology and Rheumatology, Utrecht, The Netherlands, Utrecht, Netherlands, 5Università degli Studi di Genova, Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DiNOGMI), Genoa, Italy and IRCCS Istituto Giannina Gaslini, Clinica Pediatrica e Reumatologia, Genoa, Italy, Genoa, Italy, 6Institute for Maternal and Child Health IRCCS "Burlo Garofolo," Trieste, Italy, Trieste, Italy, 7German Rheumatism Research Centre Berlin, and Charité University Medicine, Department of Rheumatology and Clinical Immunology, Berlin, Germany, Berlin, Germany, 8Ryhov County Hospital, Futurum - the Academy for health and care, Jonkoping, Sweden, Jonkoping, Sweden, 9Department of Pediatrics, University of Catania, Catania, Italy, Catania, Italy, 10Spitalul Clinic Județean De Urgenta, Tîrgu-Mures, Romania, Tîrgu-Mures, Romania, 11Paediatric Rheumatology International Trials Organisation (PRINTO), Genoa, Italy, 12IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genova, Italy, 13Università degli Studi di Genova, Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DiNOGMI), Genoa, Italy and IRCCS Istituto Giannina Gaslini, Clinica Pediatrica e Reumatologia, Genoa, Italy, Genova, Italy

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: activity score and patient outcomes, juvenile idiopathic arthritis (JIA)

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Session Information

Date: Monday, November 11, 2019

Session Title: 4M125: Pediatric Rheumatology: Outcomes & Quality of Life (1920–1925)

Session Type: ACR/ARP Abstract Session

Session Time: 4:30PM-6:00PM

Background/Purpose: The assessment of disease activity plays a pivotal role in the management of children with juvenile idiopathic arthritis (JIA). Most recent recommendations require that the parents’ and children’s perception is incorporated in the evaluation of disease course and of effectiveness of therapeutic interventions. A new disease activity tool, named parent Juvenile Arthritis Disease Activity Score (parJADAS), based only on parent-centered outcome measures, is currently under development. Aim of the study is to demonstrate, in 2 large multinational datasets, the discriminant and predictiveability of the parJADAS

Methods: The parJADAS (range 0-40) is the sum of 4 measures: 1) parent assessment of disease activity on a 21-numbered circle 0-10 VAS; 2) assessment of pain intensity on a 21-numbered circle 0-10 VAS; 3) proxy assessment of joint disease up to a maximum of 10 joints; 4) assessment of morning stiffness (MS) on a Likert scale, ranging from no MS (0 points) to > 2 hours of MS (10 points). Discriminat ability was assessed on a dataset of 8,656 children with JIA from 49 countries, enrolled in the study of Epidemiology, treatment and Outcome of Childhood Arthritis (Consolaro A et al. Lancet Child Adolesc Health. 2019), who had all the variables included in the parJADAS available. Discriminant ability was evaluated by comparing parJADAS median levels among patients with inactive disease (ID), low disease activity (LDA), moderate disease activity (MDA), and high disease activity (HDA) according to cJADAS10 and in patients whose parents were satisfied or not satisfied with disease status. To assess the influence of damage on the parJADAS, the levels of the score in patients with or without damage (Juvenile Arthritis Damage Index > 0) were compared in subjects in ID and with at least 2 years of disease course (n = 2,423). Predictive ability was assessed in a longitudinal dataset of subjects included in the PharmaChild Registry (Swart J, et al Arthritis Res Ther 2018). Patients (n = 98) were retained if they had 2 years of follow up and at least 4 visits with parJADAS available during the first year since enrolment. The AUC of the parJADAS in the first year of registry participation was calculated and compared in subjects with/without reduced functional ability (Juvenile Arthritis Functionality Scale, JAFS > 0) at 2 years and in subjects who achieved or did not achieve clinically ID at 2 years.

Results: The median levels of parJADAS in patients in ID, LDA, MDA, and HDA were 0, 3.0, 6.0, and 4.5, respectively (Kruskal-Wallis test, p < 0.001). Median parJADAS in patients whose parents were satisfied or not satisfied with disease course were 1.5 and 13.0, respectively (Mann-Whitney test p < 0.001). ParJADAS was not different in JIA patients in remission with or without damage measured with the JADI (Mann-Whitney test p = 0.08). Subjects JAFS > 0 at 2 years had greater parJADAS AUC in the first year (p < 0.001). Subjects in remission at 2 years had smaller parJADAS AUC in the first year (p < 0.001)

Conclusion: The parJADAS showed excellent discriminant and predictive ability in 2 large multinational cohorts. The score was not relevantly influenced by disease damage in JIA patients in remission.


Disclosure: F. Ridella, None; G. Januskeviciute, None; C. Trincianti, None; E. Van Dijkhuizen, None; G. Giancane, None; S. Pastore, None; K. Minden, None; M. Ekelund, None; P. Barone, None; M. Laday, None; N. Ruperto, AbbVie, 5, 8, Abbvie, 8, Ablynx, 5, 8, Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol Myers and Squibb, Eli-Lilly, EMD Serono, Glaxo Smith and Kline, 8, AstraZeneca-Medimmune, 8, Astrazeneca-Medimmune, 8, AstraZeneca-MedImmune, 5, 8, Biogen, 5, 8, BMS, Eli-Lilly, GlaxoSmithKline, F Hoffmann-La Roche, Janssen, Novartis, Pfizer, Sobi., 2, Boehringer, 8, Boehringer Ingelheim, 5, 8, Boeringher Ingelheim, 8, Bristol-Myers Squibb, 2, 5, 8, Eli Lilly, 2, 5, 8, Eli-Lilly, 8, EMD Serono, 5, 8, F Hoffmann-La Roche, 2, 5, 8, GlaxoSmithKline, 2, 5, 8, Hoffmann-La Roche, 8, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda, 8, Janssen, 2, 5, 8, Merck, 5, 8, Novartis, 2, 5, 8, Pfizer, 2, 5, 8, R-Pharma, 5, 8, SanofiServier, 5, 8, Sinergie, 5, 8, Sobi, 2, 5, 8, 9, Takeda, 5, 8; A. Ravelli, Angelini, AbbVie, Bristol-Myers Squibb, Johnson & Johnson, Novartis, Pfizer, Reckitt Benkiser, and Roche, 2, 5, 8; A. Consolaro, Abbvie, 2, Pfizer, 2.

To cite this abstract in AMA style:

Ridella F, Januskeviciute G, Trincianti C, Van Dijkhuizen E, Giancane G, Pastore S, Minden K, Ekelund M, Barone P, Laday M, Ruperto N, Ravelli A, Consolaro A. Discriminant and Predictive Ability of the Parent Version of the Juvenile Arthritis Disease Activity Score in Two Large Multination Cohorts of Patients with Juvenile Idiopathic Arthritis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/discriminant-and-predictive-ability-of-the-parent-version-of-the-juvenile-arthritis-disease-activity-score-in-two-large-multination-cohorts-of-patients-with-juvenile-idiopathic-arthritis/. Accessed March 7, 2021.
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