EXPERIENCE WITH ABATACEPT IN TREATMENT OF JUVENILE IDIOPATHIC ARTHRITIS

Persistent interest to Abatacept (ABA) keeps growing due to continuous inflow of consistent efficacy and safety data from successful clinical trials. The objective of our retrospective trial was to evaluate ABA efficacy and safety in treatment of juvenile idiopathic arthritis (JIA) in biologic-naive patients. 20 patients aged 3—17 y.o. were included into the study. All included cases had a long duration of the disease, high values of clinical and laboratory indicators of JIA activity corresponding to moderate and severe course of arthritis. 70% achieved improvement in ACR 30 after average 8 months treatment with ABA (duration range 3—20 mo). Best clinical responses were observed in patients with systemic JIA subtype (but without obvious clinical manifestations) in all 3 cases out of 3 included, and polyarticular subtype — in 9 patients out of 11. 6 patients (30,0%) discontinued treatment. Main reasons for discontinuing treatment were absence or lack of efficacy — in 4 cases, poor adherence — in 1 case, and adverse reactions — in 1 case. Hopefully these data will help practicing physicians with adequate choice of treatment

Standard and increased canakinumab dosing to quiet macrophage activation syndrome in children with systemic juvenile idiopathic arthritis

ObjectiveMacrophage activation syndrome (MAS) is a life-threatening, potentially fatal condition associated with systemic juvenile idiopathic arthritis (sJIA). Interleukin-1 (IL-1) is a key cytokine in the pathogenesis of sJIA MAS. Many cases of MAS are medically refractory to traditional doses of biologic cytokine inhibitors and may require increased dosing. When MAS occurs in the setting of sJIA treated with the IL-1 receptor antagonist (IL-1Ra), anakinra, increased anakinra dosing may be beneficial. Increased dosing of another IL-1 inhibitor, canakinumab, a monoclonal antibody to IL-1β, has not been reported to treat refractory MAS in the setting of sJIA.MethodsRetrospective data collection extracted from the electronic medical record focused on canakinumab usage and dosing in 8 children with sJIA who developed MAS at a single academic center from 2011 to 2020.ResultsEight sJIA children (five girls) with median age 8.5 years (range, 0.9–14.2 years) were included in the present study. Five children developed MAS at disease onset and three during ongoing canakinumab therapy. MAS resolved in all eight children with canakinumab treatment. When the canakinumab dosing was insufficient or MAS developed during canakinumab therapy, the dosing was temporally up-titrated (four patients, maximum 300 mg per dose) without observed side effects.ConclusionThis report provides evidence for the efficacy and safety of short-term increased doses (2–3-times normal) of canakinumab in treating sJIA associated MAS. Further study of the efficacy and safety of increased doses of canakinumab for treatment of MAS in children with sJIA is warranted

Proceedings of the 24th Paediatric Rheumatology European Society Congress: Part three

From Springer Nature via Jisc Publications Router.Publication status: PublishedHistory: collection 2017-09, epub 2017-09-0

The Use of Tocilizumab in 40 Patients With Polyarticular Juvenile Idiopathic Arthritis: the Results of a Retrospective Study

The issue of a therapy of children with juvenile idiopathic arthritis (JIA) with intolerance or insufficient effectiveness of methotrexate remains actual.Objective: Our aim was to study the efficacy and safety of tocilizumab in patients with polyarticular JIA.Methods. In a retrospective study, we studied the results of the use of tocilizumab in patients with active polyarticular JIA ( 5 active joints) resistant to prior therapy with methotrexate or a combination of methotrexate with other nonbiologic disease-modifying antiinflammatory drugs.Results. The data of 40 children (83% girls) with the onset median of polyarticular JIA of 4.8 (2.9, 8.1) years and the interval between the disease onset and the initiation of tocilizumab therapy of 5.7 (1.8, 8.5) years was analyzed. Tocilizumab was used as an intravenous infusion of 8 mg/kg (with a weight 30 kg) or 10 mg/kg (with a weight < 30 kg) every 4 weeks. The duration of tocilizumab monotherapy in 5 (13%) children was 1,109 days (452; 1,542). The stages of inactive disease (according to the criteria of C. Wallace, 2004) in 6 months of tocilizumab therapy reached 6 (15%) patients, in 42 months — 32 (80%) patients. In 3 patients, tocilizumab was canceled due to persistent remission. After 6 months of treatment, there was a marked decrease in erythrocyte sedimentation rate, C-reactive protein concentration, number of leukocytes and platelets (in all cases, p < 0.001) to normal values, which persisted throughout the whole period of drug administration. Predictors for achieving inactive disease were the initial (at the onset of tocilizumab therapy) number of peripheral blood leukocytes < 9.0X109/l [relative risk (RR) 1.92; 95% confidence interval (CI) 0.9–4.6)] and the absence of prior biological therapy (RR 1.92, 95% CI 0.9–4.6). The most frequent side effects of tocilizumab therapy were transient hypercholesterolemia (in 13), hypertriglyceridemia (in 4), transient grade II neutropenia (in 1).Conclusion. The long-term efficacy and relative safety of tocilizumab in children with polyarticular JIA have been showed

Methotrexate treatment may prevent uveitis onset in patients with juvenile idiopathic arthritis: experiences and subgroup analysis in a cohort with frequent methotrexate use

To re-evaluate the ability of methotrexate (MTX) to prevent the onset of uveitis in Russian children with juvenile idiopathic arthritis (JIA)

DIFFERENTIAL DIAGNOSIS OF SYSTEMIC-ONSET JUVENILE ARTHRITIS AND RHEUMATIC MASKS OF ONCOHEMATOLOGICAL DISEASES: A RETROSPECTIVE COHORT STUDY

Background. Patients with malignant oncohematological diseases (OHD) may have such symptoms as fever, lymphadenopathy, hepatosplenomegaly,  joint pain, arthritis, elevated erythrocyte sedimentation rate (ESR) and C-reactive  protein (CRP) concentration, anemia that require differentiation from clinical implications of systemic juvenile idiopathic arthritis (sJIA).Objective.  Our aim was to determine diagnostic criteria that can differentiate  rheumatic masks of OHD from sJIA.Methods.  The retrospective  study included 86 children with sJIA and 21 children with OHD who had rheumatic masks and were hospitalized in rheumatological departments with an initial diagnosis of sJIA. OHD were represented  by acute lymphoblastic leukemia (n = 17), neuroblastoma (n = 1), and lymphomas (n = 3).Results. Blast cells in the peripheral blood test were detected in 9/17 (53%) patients with acute leukemia at different times from the appearance of complaints and hospitalization. Diagnostic criteria for differentiating OHD from sJIA were the number of active joints  3 (diagnostic odds ratio, OR, 4.4, 95% confidence interval, CI, 1.5–13.2), CRP concentration < 15 mg/L (OR 5.6, 95% CI 1.7–18.4), platelets     307   109/L (OR 22.9, 95% CI 4.9–107.0), white blood cells     8.9   109/L (OR 50.2, 95% CI 6.3–401.3), albumin > 43.3% (OR 28.8, 95% CI 5.6–149.2),  absence of exanthema (OR 39.8, 95% CI 8.4–188.5).  The most frequent symptoms with the greatest specificity were night pain (sensitivity 0.57, specificity 1.0), bone pain (sensitivity 0.95, specificity 1.0), pathological fractures (sensitivity 0.14, specificity 1.0).Conclusion. The identified diagnostic criteria can be used for differential diagnosis of OHD with rheumatic masks and sJIA