Abstract
Background The systemic subtype of juvenile idiopathic arthritis (sJIA) can be the most severe, and unlike other forms of JIA is an autoinflammatory disease. There is evidence for the involvement of IL-1 in sJIA: treatment with the IL-1 receptor agonist, Anakinra, has shown dramatic improvement in some sJIA patients. Additionally we have shown a significant association with members of the IL-1 gene family and sJIA [1]. Caspase-1 is required to cleave IL-1β into its active form and Cryopyrin (NLRP3) is part if the IL-1β inflammasome, required for the activation of caspase-1. Mutations in NLRP3 have been found in patients that have similar clinical features with sJIA, e.g. CINCA. Here we describe a candidate gene association study of CASP1 and NLRP3 in sJIA.
Highlights
The systemic subtype of juvenile idiopathic arthritis can be the most severe, and unlike other forms of JIA is an autoinflammatory disease
15th Paediatric Rheumatology European Society (PreS) Congress Wietse Kuis, Patricia Woo, Angelo Ravelli, Hermann Girschick, Michaël Hofer, Johannes Roth, Rotraud K Saurenmann, Alberto Martini, Pavla Dolezova, Janjaap van der Net, Pierre Quartier, Lucy Wedderburn and Jan Scott Meeting abstracts – A single PDF containing all abstracts in this Supplement is available here.
We have shown a significant association with members of the IL-1 gene family and subtype of juvenile idiopathic arthritis (sJIA) [1]
Summary
The systemic subtype of juvenile idiopathic arthritis (sJIA) can be the most severe, and unlike other forms of JIA is an autoinflammatory disease. Address: University College London, London, UK * Corresponding author from 15th Paediatric Rheumatology European Society (PreS) Congress London, UK.
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