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.

Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders, including all forms of arthritis, which develops in children who are less than 16 years old. This study aimed to evaluate the clinical and laboratory features of JIA in a single center in Jordan.Methods: A retrospective analysis of the electronic medical records of pediatric patients diagnosed with JIA based on the International League of Associations for Rheumatology (ILAR) criteria during the period from 2015 to 2019 at the Pediatric Rheumatology Clinic in the Queen Rania Children’s Hospital. All patients were below the age of 14 years at the time of diagnosis and followed for at least six months. Collected data consisted of age, gender, age at initial presentation and diagnosis, JIA subtype, laboratory data, treatment options, and outcome.Results: A total of 210 patients were included in this cohort (94 males and 116 females) with the mean age at diagnosis and mean age at onset of 5.33 ± 3.40 years and 5.08 ± 3.40 years (range: 7 months – 14 years), respectively. Oligoarticular JIA was the commonest subtype (54.7%), followed by systemic arthritis (17.1%) and polyarticular arthritis (12.3%). ANA was positive in 70 patients (33.6%). Uveitis occurred in thirty (14.2%) patients.Conclusion: To the best of our knowledge, this study on this cohort is the first report on JIA in Jordan, in comparison with other regionally and internationally published reports. Oligoarticular JIA was found to be the most common subtype. For detailed knowledge on JIA characteristics and patterns, a population-based, rather than a single center study, should be conducted in Jordan.

Background Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders, including all forms of arthritis, which develops in children who are less than 16 years old. This study aimed to evaluate the clinical and laboratory features of JIA in a single center in Jordan. Methods A retrospective analysis of the electronic medical records of Pediatric patients diagnosed with JIA based on the International League of Associations for Rheumatology (ILAR) criteria during the period from 2015 to 2019 at the Pediatric Rheumatology Clinic in the Queen Rania Children’s Hospital. All patients were below the age of 14 years at the time of diagnosis and followed for at least 6 months. Collected data consisted of age, gender, age at initial presentation and diagnosis, JIA subtype, laboratory data, treatment options, and outcome. Results A total of 210 patients were included in this cohort (94 males and 116 females) with the mean age at diagnosis and mean age at onset of 5.33 ± 3.40 years and 5.08 ± 3.40 years (range: 7 months – 14 years), respectively. Oligoarticular JIA was the commonest subtype (54.7%), followed by systemic arthritis (17.1%) and polyarticular arthritis (12.3%). ANA was positive in 70 patients (33.6%). Uveitis occurred in 30 (14.2%) patients. Conclusion To the best of our knowledge, this study on this cohort is the first report on JIA in Jordan, in comparison with other regionally and internationally published reports. Oligoarticular JIA was found to be the most common subtype. For detailed knowledge on JIA characteristics and patterns, a population-based, rather than a single center study, should be conducted in Jordan.

Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders, including all forms of arthritis, begin in children who are less than 16 years old. This study aims to evaluate the clinical and laboratory features of JIA in Jordanian children in a single center.Methods: A retrospective analysis of the medical records of pediatric patients who were diagnosed as JIA based on the International League of Associations for Rheumatology (ILAR) criteria from 2015 through 2019 at a pediatric Rheumatology clinic in Queen Rania Children Hospital. All patients were below the age of 14 at the time of diagnosis. Collected data included age, gender, age at initial presentation and diagnosis, JIA subtypes, and laboratory data.Results: A total of 210 patients were included in this cohort (94 males and 116 females) with a mean age at diagnosis 5.33 ± 3.4 years and the mean age at onset of the disease was 5.08 ± 3.4 years (range 7 months – 14 years). Oligoarticular JIA was the commonest subtype (54.7%), followed by systemic arthritis (17.1%), polyarticular (12.3%). ANA was positive in 70 patients (33.6%). Uveitis occurred in thirty (14.2%) patientsConclusion: To the best of our knowledge, this cohort is the first report on JIA in Jordan, in comparison with other regional and international published reports, Oligoarticular JIA found to be the most common Subtype in our experience. To have more details about JIA characteristics, a population-based rather than a single-center study needs to be conducted in Jordan

BackgroundSystemic juvenile idiopathic arthritis (sJIA) accounts up to 15 % of all patients with JIA and is distinct from the other disease categories due to the association of articular and extraarticular manifestations. The systemic Juvenile Arthritis Disease Activity Score (sJADAS) is a composite disease activity score validated specifically for use in sJIA that includes, beside the four components of the original JADAS, a fifth item aimed to quantify the burden of systemic features. The interpretation of scores on sJADAS requires criteria that identify the states of disease activity. These criteria can be used to monitor the disease course over time and to define therapeutic targets.ObjectivesTo compare the clinical and laboratory data of each disease activity state in patients enrolled in the multinational study aimed to define the sJADAS cutoffs.MethodsData were extracted from a multinational cross-sectional dataset that included patients diagnosed as sJIA by ILAR criteria, recruited between February 2022 and November 2022. At study visit, each patient was categorized subjectively by the caring physician into one of the following disease activity states: inactive disease (ID), low (or minimal) disease activity (LDA), moderate disease activity (MDA), or high disease activity (HDA). Study data was collected through a standard case report form and entered into an electronic database.ResultsA total of 231 patients were enrolled in 29 centers in 12 countries. The mean age at diagnosis was 5.63 years. 87 patients (37.7%) were judged as having ID, 39 (16.9%) LDA, 46 (19.9%) MDA and 59 (25.5%) HDA. The comparison of the main clinical and laboratory features across patients with the four disease activity states is shown in the Table 1. Overall, the presence of extraarticular manifestations was more common in patients with MDA and HDA (P<0.00001), whereas fever, rash, hepatosplenomegaly, and lymphadenopathy were more frequent in HDA patients (<0.00001). The count of active joints increased progressively from ID to HDA (p<0.00001). The mean values of physician global assessment of disease activity and systemic manifestations, as well as the mean values of acute phase reactants, were highest in patients with HDA, with gradual decrease from MDA to LDA to ID.ConclusionThis preliminary analysis of the study data indicates that the subjective assessment of disease state by the caring physicians led to discriminate reliably patients with different level of disease activity. This evaluation will, then, serve well as reference for the subsequent analyses aimed to identify the cutoffs for the main disease activity states in sJIA.Reference[1]Tibaldi J, Pistorio A, Aldera E, et al. Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis. Rheumatology (Oxford). 2020 Nov 1;59(11):3505-3514.Table 1.Comparison of clinical and laboratory features across disease activity states (n = 231). ID=inactive disease; LDA= low (or minimal) disease activity; MDA=moderate disease activity; HDA=high disease activity; MD global VAS=physician global assessment of disease activity; MD systemic VAS= physician global assessment of systemic disease activity; ESR= erythrocyte sedimentation rate; CRP= C-reactive protein; NAJ=Number of active joints.IDLDAMDAHDANumber of patients (n, %)87 (37.7)39 (16.9)46 (19.9)59 (25.5)Age at onset, years (mean, SD)6.17 (4.24)5.44 (3.06)5.11 (3.42)5.36 (4.06)MD global VAS (mean, SD)1.08 (2.64)2.5 (2.59)5.03 (2.59)6.61 (2.91)MD systemic VAS (mean, SD)0.06 (0.23)0.88 (1.13)3.08 (2.69)7.52 (2.17)Systemic features (n,%)1 (1.1)7 (17.9)22 (47.8)56 (94.9)• Fever0 (0)0 (0)19 (41.3)55 (93.2)• Rash0 (0)4 (10.3)9 (19.6)29 (49.2)• Hepatomegaly0 (00 (0)2 (4.3)21 (35.6)• Splenomegaly0 (0)1 (2.6)4 (8.7)14 (23.7)• Lymphadenopathy1 (1.1)2 (5.1)5 (10.9)20 (33.9)• Serositis0 (0)0 (0)4 (8.7)9 (15.3)ESR, mm/h (mean, SD)8.46 (7.63)16.26 (14.43)46.11 (34.53)66.85 (32.4)CRP, mg/dl (mean, SD)0.39 (0.85)0.88 (1.93)5.17 (7.03)7.89 (6.06)NAJ > 1 (%)1 (1.1)13 (33.3)34 (73.9)53 (89.8)AcknowledgementsThis work was partially supported by the Fundación Española de Reumatología (FER).Disclosure of InterestsAna Isabel Rebollo Giménez: None declared, Yulia Vyzhga: None declared, Luca Carlini: None declared, Silvia Rosina: None declared, Elisa Patrone: None declared, Maria Katsikas: None declared, Claudia Saad-Magalhaes: None declared, Dalia El-Ghoneimy: None declared, Yasser El Miedany: None declared, Raju Khubchandani: None declared, Priyankar Pal: None declared, Gabriele Simonini Grant/research support from: educational grants from Abbvie, Sobi, Novartis, Giovanni Filocamo Consultant of: consultancies from Sobi and Novartis, Maurizio Gattinara: None declared, Fabrizio De Benedetti: None declared, Davide Montin: None declared, Adele Civino: None declared, Motasem O. Alsuweiti: None declared, Valda Stanevicha: None declared, Vyacheslav Chasnyk: None declared, Ekaterina Alexeeva Speakers bureau: speakers bureaus for Roche, Novartis and Pfizer., Grant/research support from: research grants from Roche, Pfizer, Centocor, Eli Lilly, AbbVie, Bristol-Myers Squibb, MSD, Sanofi, Amgen and Novartis., SULAIMAN AL-MAYOUF Speakers bureau: speaker fees from Sobi and Novartis, Soamarat Vilaiyuk: None declared, Angelo Ravelli Speakers bureau: from:Abbvie, Angelini, Pfizer, Novartis, BMS, Reckitt Benckiser, Sobi, Alexion, Roche.