- Meeting abstract
- Open Access
PW03-004 - PFAPA patient’s serum sensitizes monocytes to LPS
Pediatric Rheumatology volume 11, Article number: A230 (2013)
PFAPA is a pediatric auto-inflammatory syndrome of unknown etiology, characterized by recurrent fever, aphthosis, pharyngitis and cervical adenitis. Dysregulated monocyte interleukin-1 beta (IL-1β) secretion is thought to play an important role in fever flares.
We hypothesized that factor(s) present in the serum of PFAPA patients during a fever flare may induce monocytes to secrete IL-1β which prolongs symptoms.
Serum of three controls (CTRL) or PFAPA patients collected during (PFAPA-IN) and between (PFAPA-OUT) flares were incubated with monocytes isolated from healthy volunteers (n=3) and stimulated with ultra pure lipopolysaccharide (LPSup). IL-1β, TNF-α and IL-6 levels were measured by ELISA comparing serum stimulation alone and the impact of serum pre-incubation on LPSup induced cytokines.
Serum-alone treatment of monocytes did not induce any detectable increase in IL-1β, TNF-α or IL-6 secretion. However, pre-treatment with PFAPA-IN serum did result in significantly more IL-1β secretion following LPSup stimulation as compared to PFAPA-OUT serum pretreatment (330.8 ± 181.5 and 173.5 ± 100.1 pg/ml respectively; n=9; p<0.05). There was no modulation in the levels of TNF-α and IL-6 under the present conditions, arguing for a process specific for the IL-1β pathway.
Incubation of healthy donor monocytes with serum collected during a PFAPA flare increases the capacity of monocytes to secrete IL-1β through TLR4 ligation. The identity of the molecule(s) and the mechanism of action of this observation still remain to be elucidated.
Disclosure of interest
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
About this article
Cite this article
Simon, G., Busso, N., von Scheven-Gete, A. et al. PW03-004 - PFAPA patient’s serum sensitizes monocytes to LPS. Pediatr Rheumatol 11 (Suppl 1), A230 (2013). https://doi.org/10.1186/1546-0096-11-S1-A230
- Induce Cytokine
- Detectable Increase
- Recurrent Fever
- Serum Stimulation