Gattorno M et al. – Two subsets of systemic-onset juvenile idiopathic arthritis (JIA) can be identified according to patient response to IL-1 blockade. In vitro secretion of IL-1β and IL-18 by monocytes is not increased and is independent of both treatment outcome and disease activity. Methods
Aim was to assess the clinical response to IL-1 blockade and in vitro IL-1 and IL-18 secretion in pts with systemic-onset JIA
22 pts with systemic-onset JIA were treated with the IL-1 receptor antagonist (IL-1Ra) anakinra
Monocytes from 18 pts and 20 healthy donors were activated by different Toll-like receptor ligands
Intracellular and secreted IL-1 and IL-18 were analyzed by Western blotting and ELISA
Results
10 pts exhibited a dramatic response to anakinra and were classified as complete responders
11 pts had an incomplete response or no response, and 1 patient could not be classified in terms of response
Complete responders had a lower number of active joints and an increased absolute neutrophil count
In vitro IL-1 and IL-18 secretion in response to various stimuli was not increased and was independent of treatment efficacy
Likewise, secretion of IL-1Ra by monocytes from patients with systemic-onset JIA was not impaired
An overall low level of IL-1 secretion upon exposure to exogenous ATP was observed, unrelated to treatment responsiveness or disease activity
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