scholarly journals Canakinumab in patients with systemic juvenile idiopathic arthritis and active systemic features: results from the 5-year long-term extension of the phase III pivotal trials

2018 ◽  
Vol 77 (12) ◽  
pp. 1710-1719 ◽  
Author(s):  
Nicolino Ruperto ◽  
Hermine I Brunner ◽  
Pierre Quartier ◽  
Tamàs Constantin ◽  
Nico M Wulffraat ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Disease Activity Score ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Phase Iii ◽  
Activity Score ◽  
Treatment Rate ◽  
Two Phase ◽  
Link Type

ObjectivesTo evaluate the long-term efficacy and safety of canakinumab in patients with active systemic juvenile idiopathic arthritis (JIA).MethodsPatients (2–19 years) entered two phase III studies and continued in the long-term extension (LTE) study. Efficacy assessments were performed every 3 months, including adapted JIA American College of Rheumatology (aJIA-ACR) criteria, Juvenile Arthritis Disease Activity Score (JADAS) and ACR clinical remission on medication criteria (CRACR). Efficacy analyses are reported as per the intent-to-treat population.Results144 of the 177 patients (81%) enrolled in the core study entered the LTE. Overall, 75 patients (42%) completed and 102 (58%) discontinued mainly for inefficacy (63/102, 62%), with higher discontinuation rates noted in the late responders group (n=25/31, 81%) versus early responders (n=11/38, 29%). At 2 years, aJIA-ACR 50/70/90 response rates were 62%, 61% and 54%, respectively. CRACR was achieved by 20% of patients at month 6; 32% at 2 years. A JADAS low disease activity score was achieved by 49% of patients at 2 years. Efficacy results were maintained up to 5 years. Of the 128/177 (72.3%) patients on glucocorticoids, 20 (15.6%) discontinued and 28 (22%) tapered to 0.150 mg/kg/day. Seven patients discontinued canakinumab due to CR. There were 13 macrophage activation syndrome (three previously reported) and no additional deaths (three previously reported). No new safety findings were observed.ConclusionResponse to canakinumab treatment was sustained and associated with substantial glucocorticoid dose reduction or discontinuation and a relatively low retention-on-treatment rate. No new safety findings were observed on long-term use of canakinumab.Trial registration numbersNCT00886769, NCT00889863, NCT00426218 and NCT00891046.

scholarly journals Validating 10-joint juvenile arthritis disease activity score cut-offs for disease activity levels in non-systemic juvenile idiopathic arthritis

RMD Open ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. e000888 ◽  
Author(s):  
Maria Backström ◽  
Pirjo Tynjälä ◽  
Kristiina Aalto ◽  
Minna-Maija Grönlund ◽  
Heikki Ylijoki ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Disease Activity Score ◽  
Characteristic Curve ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Juvenile Arthritis ◽  
Activity Score ◽  
Inactive Disease ◽  
International Consensus ◽  
Polyarticular Disease

ObjectivesTo validate cut-offs of the Juvenile Arthritis Disease Activity Score 10 (JADAS10) and clinical JADAS10 (cJADAS10) and to compare them with other patient cohorts.MethodsIn a national multicentre study, cross-sectional data on recent visits of 337 non-systemic patients with juvenile idiopathic arthritis (JIA) were collected from nine paediatric outpatient units. The cut-offs were tested with receiver operating characteristic curve-based methods, and too high, too low and correct classification rates (CCRs) were calculated.ResultsOur earlier presented JADAS10 cut-offs seemed feasible based on the CCRs, but the cut-off values between low disease activity (LDA) and moderate disease activity (MDA) were adjusted. When JADAS10 cut-offs for clinically inactive disease (CID) were increased to 1.5 for patients with oligoarticular disease and 2.7 for patients with polyarticular disease, as recently suggested in a large multinational register study, altogether 11 patients classified as CID by the cut-off had one active joint. We suggest JADAS10 cut-off values for oligoarticular/polyarticular disease to be in CID: 0.0–0.5/0.0–0.7, LDA: 0.6–3.8/0.8–5.1 and MDA: >3.8/5.1. Suitable cJADAS10 cut-offs are the same as JADAS10 cut-offs in oligoarticular disease. In polyarticular disease, cJADAS10 cut-offs are 0–0.7 for CID, 0.8–5.0 for LDA and >5.0 for MDA.ConclusionInternational consensus on JADAS cut-off values is needed, and such a cut-off for CID should preferably exclude patients with active joints in the CID group.

scholarly journals AB0742 PREVALENCE AND RISK FACTORS OF OSTEOPOROSIS IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1400.1-1400
Author(s):  
H. Bettaieb ◽  
H. Ferjani ◽  
K. Maatallah ◽  
H. Boussaa ◽  
D. Kaffel ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Corticosteroid Therapy ◽  
Disease Activity Score ◽  
Disease Duration ◽  
Activity Score ◽  
Diagnosis Delay ◽  
Targeted Interventions ◽  
Increased Risk

Background:Childhood rheumatic diseases are associated with reduced Bone mass and increased risk of fractures (1). Several factors may interact to determine osteoporosis other than direct bone detrimental effects of the disease or its treatment.Objectives:In this work, we aimed to investigate the prevalence of bone loss in patients with JIA and to determine the relative factors associated with osteoporosis during this chronic disease.Methods:A retrospective monocentric study was carried out on JIA patients (ILAR criteria).Dual-energy x-ray absorptiometry (DEXA) was used to determine bone status. Disease activity was evaluated by JADAS10 (Juvenile Arthritis Disease Activity Score) in poly and oligoarticular subtypes and by BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in arthritis related enthesitis form. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were noted. The data were analyzed using the SPSS statistical package. A p value < 0.05 was considered significant.Results:The sample included 40 JIA (25 male and 15 female) with a mean age at disease onset of 11.3 ± 3.6 years. The median disease duration was 90 months [7-408].The median JIA diagnosis delay was 8 months [1-108]. The JIA subgroups were in decreasing order of frequency: Enthesitis-related Arthritis (n=27), Polyarticular RF- (n=4), Polyarticular RF+ (n=1), Oligoarticular (n=4), Systemic (n=2), Psoriatic Arthritis (n=1) and Undifferentiated (n=1). Median ESR and CRP were 29 mm/hour [2-98] and 14.5 mg/l [0-70] respectively. Median BASDAI score was 4.3 [1-9.7]. Median JADAS10 score was 1[1-21].Overall, 45% of patients had osteoporosis, 27.5% had osteopenia, and 27.5 % had normal bone densitometry. None of the patients had a history of vertebral or peripheral fractures.Thirty per cent of patients (n=12) were on long term corticosteroid therapy with a mean dose of 6.6 ± 2.8 mg/day. Only 12.5% (n=5) of them had a regular physical activity.Osteoporosis was associated with age at JIA onset (p=0.005), disease duration (p=0.001), ESR (p=0.08), CRP (p=0.04), BASDAI score (p=0.017) and sedentarily (p=0.026). Osteopenia was only associated with corticosteroid therapy (p=0.01). Neither osteoporosis (p=0.37) nor Osteopenia (p=0.25) was associated with disease activity score.Conclusion:In our study, osteoporosis was a common feature during JIA. A long term corticosteroid therapy and sedentarily seem to be correlated with more impaired bone abnormalities. Hence, targeted interventions are urgently required to preserve bone health during JIA.References:[1]McDonagh JE. Osteoporosis in juvenile idiopathic arthritis. Curr Opin Rheumatol. 2001;13(5):399-404.Disclosure of Interests:None declared

scholarly journals Seroprevalence of Antibodies against SARS-CoV-2 in Children with Juvenile Idiopathic Arthritis a Case-Control Study

2021 ◽  
Vol 10 (8) ◽  
pp. 1771
Author(s):  
Violetta Opoka-Winiarska ◽  
Ewelina Grywalska ◽  
Izabela Korona-Glowniak ◽  
Katarzyna Matuska ◽  
Anna Malm ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Disease Activity Score ◽  
Juvenile Arthritis ◽  
Activity Score ◽  
Control Group ◽  
Healthy Children ◽  
Serum Samples ◽  
History Of ◽  
Novel Coronavirus

There is limited data on the effect of the novel coronavirus disease (COVID-19) caused by severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) on pediatric rheumatology. We examined the prevalence of antibodies against SARS-CoV-2 in children with juvenile idiopathic arthritis (JIA) and a negative history of COVID-19 and the correlation of the presence of these antibodies with disease activity measured by juvenile arthritis disease activity score (JADAS). In total, 62 patients diagnosed with JIA, under treatment with various antirheumatic drugs, and 32 healthy children (control group) were included. Serum samples were analyzed for inflammatory markers and antibodies and their state evaluated with the juvenile arthritis disease activity score (JADAS). JIA patients do not have a higher seroprevalence of anti-SARS-CoV-2 antibodies than healthy subjects. We found anti-SARS-CoV-2 antibodies in JIA patients who did not have a history of COVID-19. The study showed no unequivocal correlation between the presence of SARS-CoV-2 antibodies and JIA activity; therefore, this relationship requires further observation. We also identified a possible link between patients’ humoral immune response and disease-modifying antirheumatic treatment, which will be confirmed in follow-up studies.

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