Abstract: Objective To investigate the changes of serum levels of galactose-de?cient IgA1 (Gd-IgA1) and nuclear factor-kappa B ((NF-κB) in children with Henoch-Schönlein purpura (HSP), and the relationships of them with inflammatory mediators. Methods A total of 70 hospitalized children with HSP were recruited from October 2014 to March 2015, and divided into general group (23 cases), gastrointestinal group (21 cases) and nephritis group (26 cases). Meanwhile, 17 healthy children were selected as control group. The serum levels of Gd-IgA1, NF-κB, IL-8, TNF-α, and ICAM-1 were detected by ELISA method. Results In the acute phase of HSP, the serum levels of Gd-IgA1, NF-κB, IL-8, TNF-α, and ICAM-1 were significantly  different among general group, gastrointestinal group, nephritis group and control group (P all < 0.01). The serum levels of Gd-IgA1, NF-κB, IL-8, TNF-α, and ICAM-1 were significantly higher in nephritis group than those in control group (P all < 0.05). In the recovery phase of HSP, the serum levels of Gd-IgA1, NF-κB, TNF-α, and IL-8 in nephritis group were still higher than those in control group (P all < 0.05), but the level of ICAM-1 was no differenct among four groups (P > 0.05). There was a positively correlation between Gd-IgA1 and NF-κB in acute phase in children with HSP (r = 0.67, P < 0.05). Conclusions Gd-IgA1 may up-regulate IL-8, TNF-α, and ICAM-1 by activating NF-κB, resulting in small vascular inflammatory injury of HSP.