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CVID Common Variable Immunodeficiency Syndrome

Summary

Common variable immunodeficiency (CVID) represents the most common immunodeficiency of the adult. CVID is a heterogeneous antibody deficiency syndrome. While central B cell development usually occurs undisturbed, peripheral antigen driven B cell differentiation into memory B cells or long lived plasma cells is often disrupted. CVID patients usually present with recurrent bacterial infections of the respiratory and gastrointestinal tract. A subgroup of patients is characterized by autoimmune manifestations, lymphoproliferation and or granulomatous inflammation. The research of our group is focused on

• a pathogenetically and clinically relevant classification of CVID
• the discovery of monogenetic defects causing CVID
• the characterization of B cell intrinsic defects of differentiation
• the characterization of defects in germinal centre formation
• the characterization of the immune dysregulation in patients with secondary disease manifestations.

The aim is a better pathogenetic understanding of the various underlying defects in order to improve diagnosis and treatment of patients with CVID.

Cooperation Partner

• EUROPADnet - European Network of primary antibody deficiency syndromes

Funding

• EUROPADnet, European FP7
• CRC620, DFG
• CCI Tandemproject, BMBF

Publications

Wehr, C., T. Kivioja, C. Schmitt, B. Ferry, T. Witte, E. Eren, M. Vlkova, M. Hernandez, D. Detkova, P. R. Bos, G. Poerksen, B. H. von, U. Baumann, S. Goldacker, S. Gutenberger, M. Schlesier, C. F. Bergeron-van der, G. M. Le, P. Debre, R. Jacobs, J. Jones, E. Bateman, J. Litzman, P. M. van Hagen, A. Plebani, R. E. Schmidt, V. Thon, I. Quinti, T. Espanol, A. D. Webster, H. Chapel, M. Vihinen, E. Oksenhendler, H. H. Peter, and K. Warnatz. 2008. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood 111:77.

Warnatz, K., and M. Schlesier. 2008. Flow cytometric phenotyping of common variable immunodeficiency. Cytometry B Clin. Cytom. 74B:261.

Goldacker, S., R. Draeger, K. Warnatz, D. Huzly, U. Salzer, J. Thiel, H. Eibel, M. Schlesier, and H. H. Peter. 2007. Active vaccination in patients with common variable immunodeficiency (CVID). Clin. Immunol. 124:294

Horn, J., V. Thon, D. Bartonkova, U. Salzer, K. Warnatz, M. Schlesier, H. H. Peter, and B. Grimbacher. 2007. Anti-IgA antibodies in Common Variable Immunodeficiency (CVID): Diagnostic workup and therapeutic strategy. Clin. Immunol. 122:152.

Bossaller, L., J. Burger, R. Draeger, B. Grimbacher, R. Knoth, A. Plebani, A. Durandy, U. Baumann, M. Schlesier, A. A. Welcher, H. H. Peter, and K. Warnatz. 2006. ICOS deficiency is associated with a severe reduction of CXCR5+CD4 germinal center Th cells. J. Immunol. 177:4927.

Warnatz, K., L. Bossaller, U. Salzer, A. Skrabl-Baumgartner, W. Schwinger, B. M. van der, J. J. van Dongen, M. Orlowska-Volk, R. Knoth, A. Durandy, R. Draeger, M. Schlesier, H. H. Peter, and B. Grimbacher. 2006. Human ICOS deficiency abrogates the germinal center reaction and provides a monogenic model for common variable immunodeficiency. Blood 107:3045.

Ferry, B. L., J. Jones, E. A. Bateman, N. Woodham, K. Warnatz, M. Schlesier, S. A. Misbah, H. H. Peter, and H. M. Chapel. 2005. Measurement of peripheral B cell subpopulations in common variable immunodeficiency (CVID) using a whole blood method. Clin. Exp. Immunol. 140:532.

Grimbacher, B., A. Hutloff, M. Schlesier, E. Glocker, K. Warnatz, R. Drager, H. Eibel, B. Fischer, A. A. Schaffer, H. W. Mages, R. A. Kroczek, and H. H. Peter. 2003. Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency. Nat. Immunol. 4:261

Groth, C., R. Drager, K. Warnatz, G. Wolff-Vorbeck, S. Schmidt, H. Eibel, M. Schlesier, and H. H. Peter. 2002. Impaired up-regulation of CD70 and CD86 in naive (CD27-) B cells from patients with common variable immunodeficiency (CVID). Clin. Exp. Immunol. 129:133.

Warnatz, K., C. Wehr, R. Drager, S. Schmidt, H. Eibel, M. Schlesier, and H. H. Peter. 2002. Expansion of CD19(hi)CD21(lo/neg) B cells in common variable immunodeficiency (CVID) patients with autoimmune cytopenia. Immunobiology 206:502.

Warnatz, K., A. Denz, R. Drager, M. Braun, C. Groth, G. Wolff-Vorbeck, H. Eibel, M. Schlesier, and H. H. Peter. 2002. Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease. Blood 99:1544.

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