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Different pattern of synthesis and secretion of IL-1beta in patients with CIAS-1 and TNFRSF1A mutations responding to IL-1 blockade


To compare the in vitro secretion of IL-1β in patients carrying CIAS-1 mutations and TRAPS patients, in an effort to understand the mechanism modulating IL-1β secretion in the different pathologies responding to anti IL-1 treatment.


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.


In healthy subjects LPS-induced IL-1β secretion was variable but consistently ≤5 ng/ml and it was markedly increased by exposure to exogenous ATP (up to 20 ng/ml). Monocytes from CINCA patients secreted abnormally elevated amounts of IL-1β after LPS stimulation (up to 40 ng/ml) that were not increased by ATP. Conversely, monocytes from TRAPS patients did not secrete more IL-1β than healthy controls in response to LPS, but similarly to CINCA patients presented a low response to ATP.


Despite a similar clinical response to anti-IL1 treatment, the pattern of IL-1β secretion of monocytes from Anakinra-responder TRAPS patients significantly differ from that observed in patients CIAS-1 mutations. This study suggests a different hierarchy in the pathogenic mechanisms leading to the inflammatory response in different diseases responsive to anti-IL-1 treatment.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Lasigliè, D., Carta, S., Tassi, S. et al. Different pattern of synthesis and secretion of IL-1beta in patients with CIAS-1 and TNFRSF1A mutations responding to IL-1 blockade. Pediatr Rheumatol 6, P212 (2008).

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  • Healthy Donor
  • Pathogenic Mechanism
  • Short Exposure
  • Anakinra
  • Elevated Amount