Autoimmunity in common variable immunodeficiency: Correlation with lymphocyte phenotype in the French DEFI study

T-Cell Abnormalities in Common Variable Immunodeficiency

“…In a study by Carbone et al [43], activated CD4 + T cells were more common in patients with suspected IgG hypercatabolism and in patients with clinical complications including lymphoid proliferation, splenomegaly, and autoimmune disease [43]. Another study showed that CVID patients with autoimmune cytopenia have increased numbers of activated CD4 + T cells, decreased numbers of naive T cells, and an increased proportion of CD21 low B cells [15]. However, Boileau et al [15] proposed that this concurrent T-and B-cell phenotypic picture is not seen in CVID patients with other autoimmune manifestations and/or patients with splenomegaly [15,46].…”

“…However, an alteration in the frequency and function of T cells has also been demonstrated in patients with CVID [12][13][14]. The influence of these defects on the interaction between T and B cells could explain not only defective antibody production, but also the development of other complications, including recurrent bacterial and viral infections, gastrointestinal disease, lymphoma, autoimmunity, inflammation, and early-onset bronchiectasis in CVID patients [15][16][17]. In this review, we describe phenotypic and functional T-cell defects in patients with CVID and review the literature on the effects of intravenous immunoglobulin (IVIG) infusion on T-cell components in CVID.…”

“…Another study showed that CVID patients with autoimmune cytopenia have increased numbers of activated CD4 + T cells, decreased numbers of naive T cells, and an increased proportion of CD21 low B cells [15]. However, Boileau et al [15] proposed that this concurrent T-and B-cell phenotypic picture is not seen in CVID patients with other autoimmune manifestations and/or patients with splenomegaly [15,46]. Overall, according to recent findings on the correlation between CD21 low B cells in CVID and autoimmunity (such as autoimmune cytopenia and rheumatoid arthritis) [15,46], a restricted B-cell subset and help from activated CD4…”

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

“…More than 25% of CVID patients have autoimmune complications which are poorly understood and in some cases that are difficult to manage (1,6,7). The pathogenesis of autoimmunity in CVID is an astonishing paradox: autoantibodies and auto-reactive B cells can be detected in patients, even if serum immunoglobulins are very low and specific response to antigens is impaired.…”

“…Sometimes ITP and AHA coexist in Evans Syndrome. In CVID patients, cytopenia is definitely associated with an increased frequency of splenomegaly, however, hypersplenism is unable to entirely explain this association and the physiopathological link remains unclear (6). In order to minimize the risk of severe infections, splenectomy should be avoided in CVID patients, even in the presence of cytopenia, however this recommendation is not universally accepted.…”