ABSTRACT
The receptor for advanced glycation end products (RAGE) has been studied
in several respiratory diseases described as an important inflammatory
mediator. The RAGE-axis is activated by multiple endogenous ligands
related to pro-inflammatory states, upregulate the RAGE expression. The
function of soluble RAGE (sRAGE) is not completely understood, it has
been hypothesized an anti-inflamatory role as RAGE decoy receptor.
Few studies have explored the RAGE-axis in Cystic Fibrosis (CF) with
contradictory results. Based on previously, we present this pilot study
with the aim of describe the plasma sRAGE levels in children with cystic
CF (CFp), compare with the sRAGE levels in a healthy cohort and study
its possible correlation with CFp clinical features.
We conducted a single-center, cross-sectional observational study. We
included 35 clinically stable CF patients (aged < 18 years).
The median plasma sRAGE level in CFp was 1494,75 pg/ml [interquartile
range (IQR) 708,75pg/ml], compared with 714,20 pg/ml (IQR) in the
historical cohort of healthy controls (p < 0,001). A positive
correlation was found between plasma sRAGE level and forced expiratory
volume in 1 second/forced vital capacity ratio (FEV1/FVC) (p 0,004) and
forced expiratory flow between 25% and 75% (FEF25%-75%) (p 0,032).
In this preliminary study, the plasma sRAGE level were higher in CFp
than in healthy controls. Also, we described a positive correlation
between FEV1/FVC and FEF25%-75% and plasma sRAGE. To our knowledge,
our study is the largest to describe plasma sRAGE values in CFp and
the only one carried out in pediatric CF population.