Systemic Lupus Erythematosus as Manifestation of Common Variable Immunodeficiency Disorder

Abstract

Common variable immunodeficiency disorder (CVID) is a primary immunodeficiency (PID) with heterogeneous clinical features. It is linked to different autoimmune diseases from autoimmune cytopenias to rheumatologic diseases such as systemic lupus erythematosus (SLE). Patients with the co-occurrence of SLE and CVID are infrequent in the literature and represent less than 1% from the overall. The objective of this review is to determine the clinical and demographic characteristics presented for this group of patients. In addition, it has been explored the similarities and differences that both pathologies have, because they represent opposite poles. SLE is characterized by a polyclonal activation of B-cells with hyperproduction of autoantibodies (hypergammaglobulinemia), by contrary CVID is a PID defined by a hypogammaglobulinemia with deficiencies of IgG and/or IgM and IgA immunoglobulins. Thus, the therapeutic approach of both pathologies is totally the opposite.