Abstract: Objective　To explore the association of galactose-deficient IgA1 levels with clinical features, and further to provide guidance for individualized treatment of HSP. Methods According to the clinical symptoms and curative effect, 57 children with HSP were divided into four groups: non-HSPN group (n=26), HSPN group (n=7), refractory HSP group (n=7) and remission group (n=17). In non-HSPN group, 12 cases received glucorticoid therapy and 14 cases did not. Serum galactose-deficient IgA1 (Gd-IgA1) concentrations were detected using a Helix aspersa-lectin-based enzyme-linked immunosorbent assay (ELISA), and the total IgA1 levels were measured by ELISA. Results The serum Gd-IgA1 level was significantly higher in 40 HSP children who were not cured than that in remission group and control group (P<0.05). However, there was no difference in Gd-IgA1 level between remission group and control group (P>0.05). Compared with the control group, the serum Gd-IgA1 level was significantly higher in HSPN group, non-HSPN group and refractory HSP, and children with refractory HSP had significantly higher Gd-IgA1 level than children in non-HSPN group (P<0.05). No significant difference in Gd-IgA1 level was found either between HSPN group and refractory HSP group or between HSPN group and non-HSPN group (P>0.05). Furthermore, in non-HSPN group, the serum Gd-IgA1 level in HSP children who were not treated with glucorticoid was significantly higher than that in HSP children treated with glucorticoid (P<0.05). Conclusions The serum Gd-IgA1 level is associated with the disease activity and curative effect of HSP, especially in children with refractory HSP, and it is thus likely to be a new non-invasive disease activity marker for guiding the proper usage of glucocorticoid and immunosuppressants in HSP children.