Autoimmunity in Common Variable Immunodeficiency

Overview

Journal J Clin Immunol

Publisher Springer

Specialty Allergy & Immunology

Date 2008 Apr 30

PMID 18443901

Citations 14

Authors

Susana Lopes-da-Silva

Luiz Vicente Rizzo

Affiliations

Soon will be listed here.

Abstract

Background: Autoimmunity has been increasingly recognized as a major issue in patients with common variable immunodeficiency (CVID), the most common symptomatic primary immunodeficiency in adulthood. Different authors report high prevalences of autoimmune diseases in CVID, and several mechanisms have been proposed to explain this apparent paradox. Genetic predisposition, under current surveillance, innate and adaptive immunity deficiencies leading to persistent/recurrent infections, variable degrees of immune dysregulation, and possible failure in central and peripheral mechanisms of tolerance induction or maintenance may all contribute to increased autoimmunity.

Conclusions: Data on the clinical/immunological profile of affected patients and treatment are available mostly concerning autoimmune cytopenias, the most common autoimmune diseases in CVID. Treatment is based on conventional alternatives, in association with short experience with new agents, including rituximab and infliximab. Benefits of early immunoglobulin substitutive treatment and hypothetical premature predictors of autoimmunity are discussed as potential improvements to CVID patients' follow-up.

Citing Articles

Decoding mutational hotspots in human disease through the gene modules governing thymic regulatory T cells.

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Assessment of autoantibodies in paediatric population with primary immunodeficiencies: a pilot study.

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The Dysfunctional Immune System in Common Variable Immunodeficiency Increases the Susceptibility to Gastric Cancer.

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IL-10-Producing Regulatory B Cells Are Decreased in Patients with Common Variable Immunodeficiency.

Silveira Barsotti N, Almeida R, Costa P, Barros M, Kalil J, Kokron C PLoS One. 2016; 11(3):e0151761.

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Clinical Features, Non-Infectious Manifestations and Survival Analysis of 161 Children with Primary Immunodeficiency in Mexico: A Single Center Experience Over two Decades.

Lugo Reyes S, Ramirez-Vazquez G, Cruz Hernandez A, Medina-Torres E, Ramirez-Lopez A, Espana-Cabrera C J Clin Immunol. 2015; 36(1):56-65.

PMID: 26707787 DOI: 10.1007/s10875-015-0226-5.

References

Kanai T, Watanabe M . Clinical application of human CD4+ CD25+ regulatory T cells for the treatment of inflammatory bowel diseases. Expert Opin Biol Ther. 2005; 5(4):451-62. DOI: 10.1517/14712598.5.4.451. View

De Vera M, Al-Harthi L, Gewurz A . Assessing thymopoiesis in patients with common variable immunodeficiency as measured by T-cell receptor excision circles. Ann Allergy Asthma Immunol. 2004; 93(5):478-84. DOI: 10.1016/S1081-1206(10)61416-0. View

Sneller M, Strober W . Abnormalities of lymphokine gene expression in patients with common variable immunodeficiency. J Immunol. 1990; 144(10):3762-9. View

Holm A, Aukrust P, Aandahl E, Muller F, Tasken K, Froland S . Impaired secretion of IL-10 by T cells from patients with common variable immunodeficiency--involvement of protein kinase A type I. J Immunol. 2003; 170(11):5772-7. DOI: 10.4049/jimmunol.170.11.5772. View

Cunningham-Rundles C, Radigan L . Deficient IL-12 and dendritic cell function in common variable immune deficiency. Clin Immunol. 2005; 115(2):147-53. DOI: 10.1016/j.clim.2004.12.007. View

Jiang H, Chess L . An integrated view of suppressor T cell subsets in immunoregulation. J Clin Invest. 2004; 114(9):1198-208. PMC: 524238. DOI: 10.1172/JCI23411. View

Michel M, Chanet V, Galicier L, Ruivard M, Levy Y, Hermine O . Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of the literature. Medicine (Baltimore). 2004; 83(4):254-263. DOI: 10.1097/01.md.0000133624.65946.40. View

Zhang L, Radigan L, Salzer U, Behrens T, Grimbacher B, Diaz G . Transmembrane activator and calcium-modulating cyclophilin ligand interactor mutations in common variable immunodeficiency: clinical and immunologic outcomes in heterozygotes. J Allergy Clin Immunol. 2007; 120(5):1178-85. PMC: 2908504. DOI: 10.1016/j.jaci.2007.10.001. View

Bryant A, Calver N, Toubi E, Webster A, Farrant J . Classification of patients with common variable immunodeficiency by B cell secretion of IgM and IgG in response to anti-IgM and interleukin-2. Clin Immunol Immunopathol. 1990; 56(2):239-48. DOI: 10.1016/0090-1229(90)90145-g. View

10.

Rump J, Jahreis A, Schlesier M, Drager R, Melchers I, Peter H . Possible role of IL-2 deficiency for hypogammaglobulinaemia in patients with common variable immunodeficiency. Clin Exp Immunol. 1992; 89(2):204-10. PMC: 1554446. DOI: 10.1111/j.1365-2249.1992.tb06933.x. View

11.

Vlkova M, Thon V, Sarfyova M, Blaha L, Svobodnik A, Lokaj J . Age dependency and mutual relations in T and B lymphocyte abnormalities in common variable immunodeficiency patients. Clin Exp Immunol. 2006; 143(2):373-9. PMC: 1809591. DOI: 10.1111/j.1365-2249.2006.02999.x. View

12.

Alachkar H, Taubenheim N, Haeney M, Durandy A, Arkwright P . Memory switched B cell percentage and not serum immunoglobulin concentration is associated with clinical complications in children and adults with specific antibody deficiency and common variable immunodeficiency. Clin Immunol. 2006; 120(3):310-8. DOI: 10.1016/j.clim.2006.05.003. View

13.

Geha R, Notarangelo L, Casanova J, Chapel H, Conley M, Fischer A . Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol. 2007; 120(4):776-94. PMC: 2601718. DOI: 10.1016/j.jaci.2007.08.053. View

14.

Taubenheim N, von Hornung M, Durandy A, Warnatz K, Corcoran L, Peter H . Defined blocks in terminal plasma cell differentiation of common variable immunodeficiency patients. J Immunol. 2005; 175(8):5498-503. DOI: 10.4049/jimmunol.175.8.5498. View

15.

Schaffer A, Salzer U, Hammarstrom L, Grimbacher B . Deconstructing common variable immunodeficiency by genetic analysis. Curr Opin Genet Dev. 2007; 17(3):201-12. DOI: 10.1016/j.gde.2007.04.002. View

16.

Brouet J, Chedeville A, Fermand J, Royer B . Study of the B cell memory compartment in common variable immunodeficiency. Eur J Immunol. 2000; 30(9):2516-20. DOI: 10.1002/1521-4141(200009)30:9<2516::AID-IMMU2516>3.0.CO;2-Z. View

17.

Diamond B . Autoimmunity. Immunol Rev. 2005; 204:5-8. DOI: 10.1111/j.0105-2896.2005.00258.x. View

18.

Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T, Eren E . The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood. 2007; 111(1):77-85. DOI: 10.1182/blood-2007-06-091744. View

19.

Bates C, Ellison M, Lynch D, Cool C, Brown K, Routes J . Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency. J Allergy Clin Immunol. 2004; 114(2):415-21. DOI: 10.1016/j.jaci.2004.05.057. View

20.

Rump J, Jahreis A, Schlesier M, Stecher S, Peter H . A double-blind, placebo-controlled, crossover therapy study with natural human IL-2 (nhuIL-2) in combination with regular intravenous gammaglobulin (IVIG) infusions in 10 patients with common variable immunodeficiency (CVID). Clin Exp Immunol. 1997; 110(2):167-73. PMC: 2265503. DOI: 10.1111/j.1365-2249.1997.tb08313.x. View