Accumulating evidence indicates that HMGB1 is closely involved in fibrotic diseases, including lung fibrosis, cystic fibrosis, liver fibrosis and pulmonary fibrosis (He et al., 2007; Hamada et al., 2008; Rowe et al., 2008; Tu et al., 2012), whereas the inhibition of HMGB1 signaling can act against experimental models of fibrotic disorders (Ge et al., 2011; Entezari et al., 2012). This evidence concerns the gene HMGB1 and cystic fibrosis.