Systemic Lupus Erythematosus as Manifestation of Common Variable Immunodeficiency Disorder

CVID (common variable immunodeficiency disorder) is a primary immunodeficiency disorder (PID) with a wide range of clinical symptoms. It has been related to a variety of autoimmune disorders, including autoimmune cytopenias and rheumatologic conditions like systemic lupus erythematosus (SLE). Patients with SLE and CVID co-existing are uncommon in the literature, accounting for less than 1% of the total population. The aim of this study is to find out what clinical and demographic characteristics this group of patients has. Furthermore, since both pathologies reflect opposite poles, the similarities and distinctions between them have been investigated. SLE is defined by polyclonal activation of B cells and hyperproduction of autoantibodies (hypergammaglobulinemia), while CVID is defined by hypogammaglobulinemia and deficiencies of IgG, IgM, and IgA immunoglobulins. As a result, the treatment approaches for both pathologies are diametrically opposed.

Author (s) Details

Carmen M. Cabrera
Immunology Section, University General Hospital of Ciudad Real, Spain and Faculty of Medicine of Ciudad Real, University of Castilla-La Mancha, Spain.

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