CVID is the most frequent symptomatic primary antibody deficiency in adulthood characterized by the marked reduction of IgG and IgA serum levels. Successful use of polyvalent immunoglobulin replacement therapy is effective to treat and prevent recurrent infections, that have progressively become the major cause of morbidity and mortality in CVID patients. The management is particularly challenging, therefore it requires multiple lines of immunosuppressive treatments. Over the last years, the anti-CD20 monoclonal antibody has been increasingly used for the treatment of both autoimmune and non-malignant lymphoproliferative manifestations associated with CVID.

This study provides evidence on the use of rituximab in CVID. First of all the mechanisms were described for the rituximab mediated B-cell depletion; then, the literature data were analyzed regarding the CVID-related complications for which rituximab has been used, focusing on autoimmune cytopenias, GLILD, and non-malignant lymphoproliferative syndromes.

The study concluded through its findings that in the vast majority of the studies, rituximab has proven to be an effective and relatively safe therapeutic option. However, there are currently no data on the long-term efficacy and side effects of rituximab and other second-line therapeutic options.