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

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