IgG4-related disease (IgG4-RD) is a new group of diseases discovered in recent years and was unified in 2010 as IgG4-RD. Its clinical features include elevated serum IgG4 levels; limited or diffuse enlargement, nodules, masses, and wall thickening in one or more organs on imaging; lymphocyte and IgG4+ plasma cell infiltration with fibrosclerotic changes. The spectrum of IgG4-related diseases includes: IgG4-associated salpingitis (Mikulicz disease, MD), autoimmune pancreatitis, inflammatory pseudotumor, retroperitoneal fibrosis, autoimmune hepatitis, and sclerosing cholangitis. In addition to significantly elevated serum IgG, laboratory tests may also show hyperimmunoglobulinemia, proteinuria, positive ANA and positive RF, which need to be differentiated from other rheumatic diseases. After the symptoms are relieved, immunosuppressants can be added to reduce the hormone dosage or maintain the remission, and the specific plan should be combined with the patient’s clinical performance to achieve individualized treatment.