Even though glucocorticoid is the current first line medication for IgG4-RD, it is well
accepted in the field that excessive dosage of GC, especially accumulative dosage, is
associated with increasing organ damage. Although B cell depletion with rituximab has been
verified to be an effective treatment for IgG4-RD, even without concomitant GC therapy,
rituximab can increase the risk of infection during the treatment. Belimumab is an
IgG1-lambda monoclonal antibody that prevents the survival of B lymphocytes by blocking the
binding of soluble human B lymphocyte stimulator protein (BLyS) to receptors on B
lymphocytes. Previous studies and trails suggested that the activity of B-cell mediated
immunity and autoimmune responses were ameliorated after belimumab without increasing rates
of adverse events when compared to standard of care . However, the efficacy and tolerability
of belimumab in IgG4-RD patients have not been examined before. This randomized, control
clinical trial aimed to evaluate the tolerability and the efficacy of Belimumab for
maintenance treatment for IgG4-RD.