The Hindi version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Hindi language. The reading comprehension of the questionnaire was tested in ten JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach\u2019s alpha, interscale correlations, test\u2013retest reliability, and construct validity (convergent and discriminant validity). A total of 275 JIA patients (28.4% systemic, 10.9% oligoarticular, 13.8% RF negative polyarthritis, 46.9% other categories) and 98 healthy children were enrolled in three centres. The JAMAR components discriminated well healthy subjects from JIA patients. Notably, there is no significant difference between the healthy subjects and their affected peers in the school-related problems variable. All JAMAR components revealed good psychometric performances. In conclusion, the Hindi version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research

DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

Peer reviewe

American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus

10.1002/acr.23834ARTHRITIS CARE & RESEARCH715579-59

DEVELOPMENT AND INITIAL VALIDATION OF THE SYSTEMIC JADAS, A NEW COMPOSITE DISEASE ACTIVITY SCORE FOR SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS

Background: Juvenile Arthritis Disease Activity Score (JADAS) has gained increasing popularity for the measurement of the level of disease activity in patients with juvenile idiopathic arthritis (JIA). However, so far the JADAS has been validated only in children with the non-systemic categories of JIA. Objectives: To develop and validate the systemic JADAS (sJADAS), a new version of the JADAS specific to systemic JIA (sJIA). Methods: The sJADAS is made up by adding a fifth item, named Systemic Manifestation Score (SMS), to the four items included in the original tool (physician global assessment of disease activity, parent/patient global assessment of well-being, active joint count and erythrocyte sedimentation rate). The SMS ranges from 0 to 10 and is aimed to quantify the activity of systemic features. The sJADAS score ranges from 0 to 50. The validation sample included patients with definite and possible/probable sJIA with active systemic manifestations, which should comprise fever, who were assessed at baseline and then at a subsequent visit, 2 weeks to 3 months after initial evaluation. Validation procedures included assessment of concurrent, construct and discriminant validity, internal consistency and responsiveness to clinical change. Results: A total of 161 patients, 86.7% (n=137) with definite sJIA and 13.3% (n=21) with possible/probable sJIA, assessed at disease onset (n=90; 56.6%) or at time of a disease flare (n=69; 43.4%), were enrolled at 57 centers in 10 countries from February 2017 to December 2018. Median age at disease onset was 5.0 years (interquartile range, IQR 2.8 - 8) and median age at study entry was 6.9 years (IQR 3.8–10.8). Median disease duration from onset to study entry was 0.2 years (IQR 0.1–1.9). The median sJADAS at baseline visit was 28.2 (IQR 22.8– 35.0). The sJADAS correlated strongly with JADAS10 (rs=0.98) and clinical JADAS10 (cJADAS10) (rs=0.91); moderately with functional ability scales (JAFS and CHAQ) (rs=0.69; rs=0.62) and total score (rs=0.57) and physical (rs=0.60) and psychosocial (rs=0.40) subscale scores of the health-related quality of life tool (PRQL) and with pain VAS (rs=0.57); and mildly with the CRP (rs=0.42). The sJADAS discriminated well between patients with or without morning stiffness (p<0.0001), with different levels of disease activity defined subjectively by the physician (p<0.0001) and with different degrees of pain (p<0.0001). The internal consistency was good (Cronbach’s alpha=0.64) and comparable to that of JADAS10 (Cronbach’s alpha=0.59,) and cJADAS10 (Cronbach’s alpha=0.63). Responsiveness to change, measured on all patients (SRM=2.23) and on patients classified as improved at second visit (SRM=2.41), was strong and superior to that of JADAS10 (SRM=1.92 and 2.14, respectively). Conclusion: The sJADAS was found to be a valid instrument for the assessment of disease activity in sJIA. This composite score is feasible and easily applicable in standard clinical practice, which should result in its widespread acceptance and use. The strong responsiveness to clinical change over time indicates that the sJADAS is suitable to assess therapeutic response in sJIA clinical trials

Increased incidence of inflammatory bowel disease on etanercept in juvenile idiopathic arthritis regardless of concomitant methotrexate use

Objectives: To describe risk factors for inflammatory bowel disease (IBD) development in a cohort of children with juvenile idiopathic arthritis (JIA). Methods: JIA patients who developed IBD were identified from the international Pharmachild register. Characteristics were compared between IBD and non-IBD patients and predictors of IBD were determined using multivariable logistic regression analysis. Incidence rates of IBD events on different disease-modifying anti-rheumatic drugs (DMARDs) were calculated, differences between therapies were expressed as relative risks (RR). Results: Out of 8,942 patients, 48 (0.05%) developed IBD. These were more often male (47.9% vs 32.0%) and HLA-B27 positive (38.2% vs 21.0%) and older at JIA onset (median 8.94 vs 5.33 years) than patients without IBD development. They also had more often a family history of autoimmune disease (42.6% vs 24.4%) and enthesitis-related arthritis (ERA) (39.6% vs 10.8%). The strongest predictors of IBD on multivariable analysis were ERA (OR: 3.68, 95% CI: 1.41-9.40) and a family history of autoimmune disease (OR: 2.27, 95% CI: 1.12-4.54). Compared with methotrexate monotherapy, the incidence of IBD on etanercept monotherapy (RR: 7.69, 95% CI: 1.99-29.74), etanercept with methotrexate (RR: 5.70, 95% CI: 1.42-22.77) and infliximab (RR: 7.61, 95% CI: 1.27-45.57) therapy was significantly higher. Incidence on adalimumab was not significantly different (RR: 1.45, 95% CI: 0.15-13.89). Conclusion: IBD in JIA was associated with ERA and a family history of autoimmune disease. An increased IBD incidence was observed for etanercept therapy regardless of concomitant methotrexate use

Increased incidence of inflammatory bowel disease on etanercept in juvenile idiopathic arthritis regardless of concomitant methotrexate use

To describe risk factors for IBD development in a cohort of children with JIA