Different Pattern of Synthesis and Secretion of IL-1beta in Patients with CIAS-1 and TNFRSF1A Mutations Responding to IL-1 Blockade

Methods Monocytes from 6 CINCA and 4 TRAPS patients selected for treatment with Anakinra were activated with 1 μg/ml of LPS for 3 hours, at baseline and after 7 days from the beginning of the treatment. For comparison, monocytes from 24 healthy donors were also studied. Intracellular pro-IL-1β and secreted IL-1β were analysed by Western blotting and ELISA before and after a short exposure (15 min) to exogenous ATP that accelerates IL-1β secretion.