Abstract
Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one
Highlights
Periodic fever syndromes (PFS) are rare autoinflammatory conditions that include, among others, cryopyrinassociated periodic syndromes (CAPS), familial Mediterranean fever (FMF), hyper-IgD syndrome/mevalonate kinase deficiency (HIDS/Mevalonate Kinase Deficiency (MKD)), TNF receptor-associated periodic syndrome (TRAPS) [1]
Primary objective was to demonstrate that CAN 150 mg sc q4w is superior to placebo (PBO) in achieving a clinically meaningful response defined as resolution of the index flare at Day and no new disease flares over weeks of treatment
To evaluate the results obtained by the application of Next-generation sequencing (NGS) in a cohort of patients affected by undefined AIDsevaluated at our center Methods: In this study we enrolled 242 patients evaluated at our center from 2010 to march 2016 affected by undefined autoinflammatory diseases (AID)
Summary
Periodic fever syndromes (PFS) are rare autoinflammatory conditions that include, among others, cryopyrinassociated periodic syndromes (CAPS), familial Mediterranean fever (FMF), hyper-IgD syndrome/mevalonate kinase deficiency (HIDS/MKD), TNF receptor-associated periodic syndrome (TRAPS) [1]. Objectives: The aim of this study is to investigate the clinical significance of serum IL-18 levels as a marker for disease activity and for predicting the prognosis in patients with s-JIA during TCZ therapy. Methods: We serially measured serum IL-18 levels in 14 patients with s-JIA during TCZ therapy and compared them with the disease activity and their prognosis. Conclusion: Serum IL-18 level is a useful marker for assessing the disease activity and predicting the prognosis in patients with s-JIA during TCZ therapy. Objectives: To test our hypothesis, we analyzed serum levels of cytokines in patients with both diseases and compared them with the clinical features. Injection of canakinumab, a monoclonal anti-IL-1ß antibody with prolonged plasma half life, completely suppressed disease activity in a toddler with systemic JIA and normalized inflammatory parameters and biomarkers This treatment clearly showed advantage over daily sq. The disease had polycyclic course in 2/3 of patients and remission off treatment was achieved in 1/3 of cases
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