Abstract:Objective To systematically evaluate the efficacy and safety of steroid combined with immunosuppressants in the treatment of primary IgA nephropathy in children. Methods English and Chinese electronic databases were searched to include the studies on the efficacy and safety of steroid combined with immunosuppressants versus steroid alone in the treatment of primary IgA nephropathy in children. Outcome measures included proteinuria remission rate, urinary protein quantification, incidence of adverse events, estimated glomerular filtration rate, and incidence of renal dysfunction. Review Manager 5.3 software was used for data analysis. Results A total of 7 studies with 381 children were included. The children had moderate to severe proteinuria. The Meta analysis showed that compared with the steroid alone group, the steroid combined with immunosuppressants group achieved a significantly higher rate of proteinuria remission (RR=1.36, 95% CI: 1.19-1.55, P < 0.001) and significantly lower urinary protein quantification (SMD=-0.82, 95% CI: -1.23 to -0.41, P < 0.001). There was no significant difference in the incidence rate of adverse events between the two groups (RR=1.28, 95% CI: 0.92-1.77, P=0.14). Conclusions The current evidence shows that for children with primary IgA nephropathy who have moderate to severe proteinuria, steroid combined with immunosuppressants has a better effect than steroid alone and does not increase the incidence rate of adverse events.