In our study, IgG4-RD patients responded well to GCs plus CYC or MMF, and a majority of them experienced marked improvements within a month of treatment, which were manifested as improvement in the affected organs; reductions in the concentrations of serum ESR, CRP, IgG, and IgG4; as well as successful GC tapering. Here, ESR1 is linked to immunoglobulin G4-related sclerosing disease.