Filling the Gap: Toward a Disease Activity Tool for Systemic Juvenile Idiopathic Arthritis

Minoia, Francesca; Consolaro, Alessandro; Ravelli, Angelo

doi:10.3899/jrheum.170703

Cited by 4 publications

(2 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These criteria would correspond to no less than the state of low (or minimal) disease activity 26,27,28 , which is regarded as a qualified therapeutic target in patients with JIA 29 . Note that a disease activity tool specific to sJIA is not yet available 30 . We did not assess therapeutic effectiveness in percentage improvement in clinical signs and symptoms because current clinical practice mandates good overall disease control 31 .…”

Section: Discussionmentioning

confidence: 99%

Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis

Saccomanno

Tibaldi²,

Minoia³

et al. 2019

J Rheumatol

Self Cite

View full text Add to dashboard Cite

Objective.To seek predictors of therapeutic response to the interleukin (IL)-1 inhibitor anakinra in children with systemic-onset juvenile idiopathic arthritis (sJIA).Methods.The clinical charts of all patients with sJIA who were newly treated with anakinra at our center between 2004 and 2017 were reviewed retrospectively. Predictors included baseline demographic, clinical, and laboratory variables as well as previous or concomitant therapies. The effectiveness of anakinra was assessed at 1 year after treatment start. Complete clinical response (CCR) was defined as absence of fever, physician’s global assessment ≤ 1, count of active joints ≤ 1, negative C-reactive protein, and ≥ 75% reduction of corticosteroid dose. According to the intention-to-treat principle, patients who had anakinra discontinued before 1 year for any reasons other than disease remission were classified as nonresponders. Statistics included univariate and multivariable analyses.Results.Of the 62 patients included in the study, 24 (39%) met the criteria for CCR at 1 year, whereas 38 (61%) did not. On multivariable analysis, independent correlations with achievement of CCR were identified for shorter disease duration, lower active joint count, higher ferritin level, and greater activity of systemic manifestations. The area under the curve of the model was 0.83.Conclusion.Our findings help to delineate the clinical profile of patients with sJIA who are more likely to benefit from IL-1 blockade. They also underscore the need for studies aimed at examining the therapeutic role of early IL-1 inhibition and to identify biomarkers predicting response to either IL-1 or IL-6 antagonists.

show abstract

Section: Discussionmentioning

confidence: 99%

Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis

Saccomanno

Tibaldi²,

Minoia³

et al. 2019

J Rheumatol

Self Cite

View full text Add to dashboard Cite

show abstract

“…At present, clinical measurement tools specifically validated for use in SJIA patients are lacking. 39 In randomized controlled trials evaluating drug efficacy in SJIA patients, the adapted pediatric ACR 30/50 was used for measuring therapeutic response. Resolution of fever (≥38°C) during the week preceding the evaluation or the absence of fever in the previous 2 weeks and the reduction of the systemic GCS dose by at least 10% from the baseline dose were added to six core set variables.…”

Section: Sjiamentioning

confidence: 99%

The role of IL-1 inhibition in systemic juvenile idiopathic arthritis: current status and future perspectives

Toplak¹,

Blazina²,

Avčin³

2018

DDDT

View full text Add to dashboard Cite

The pathogenesis, clinical course, and response to treatment in systemic juvenile idiopathic arthritis (SJIA) differ from other types of juvenile idiopathic arthritis and are similar to other interleukin-1 (IL-1)-mediated diseases. The main cytokine involved in the pathogenesis of SJIA is IL-1β, which can be neutralized by targeted anti-IL-1 therapy. In SJIA, no antibodies have been found and there is growing evidence that it is mainly an autoinflammatory and not an autoimmune disease. Before the era of biologic therapy, treatment of SJIA was primarily based on long-term treatment with high doses of glucocorticosteroids (GCS). The side effects of GCS could have a significant impact on the outcome of the disease and could cause long-term damage. Treatment with anti-IL-1 agents early in the disease course has revolutionized the management principles of SJIA. However, not all SJIA patients respond equally well to anti-IL-1 therapy, and it has been shown that age at the onset of disease, duration of the disease, number of affected joints, neutrophil count, and ferritin level can predict the response to anti-IL-1 therapy. In particular, an elevated ferritin level should prompt testing for macrophage activation syndrome (MAS), the most severe complication of SJIA. Anti-IL-1 therapy has been shown to be effective also in patients with MAS. Although anti-IL-1 agents are currently not recommended as first-line treatment, there is growing evidence that anti-IL-1 agents introduced at the beginning of SJIA could enable lower doses and a shorter duration of GCS therapy, change the long-term disease outcome, and even influence molecular disease patterns. There are currently three anti-IL-1 agents available: anakinra, canakinumab, and rilonacept. In this review, we present the current knowledge on the pathogenesis of SJIA, the rational for anti-IL-1 treatment, and future perspectives on the treatment of SJIA.

show abstract

Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis

et al. 2020

View full text Add to dashboard Cite

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.

show abstract

Filling the Gap: Toward a Disease Activity Tool for Systemic Juvenile Idiopathic Arthritis

Cited by 4 publications

References 18 publications

Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis

Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis

The role of IL-1 inhibition in systemic juvenile idiopathic arthritis: current status and future perspectives

Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis

Contact Info

Product

Resources

About