Journal of Clinical Immunology

, Volume 28, Supplement 1, pp 42–45

Autoimmune Manifestations in Common Variable Immunodeficiency

Article

DOI: 10.1007/s10875-008-9182-7

Cite this article as:
Cunningham-Rundles, C. J Clin Immunol (2008) 28(Suppl 1): 42. doi:10.1007/s10875-008-9182-7

Abstract

Introduction

About 20% of subjects with common variable immune deficiency (CVID) develop an autoimmune complication, most often immune thrombocytopenia or hemolytic anemia. While the pathogenesis of autoreactivity is unknown for CVID subjects in general, and to a greater extent in those with autoimmunity, there is a loss of switched memory B cells.

Discussion

About 7–8% of CVID subjects have mutations in the transmembrane activator and calcium-modulating cyclophilin ligand interactor (TACI), a significant association with this immune defect, although the same mutations may be found in normal relatives and rarely in healthy blood donors. In addition to generalized B cell dysfunction, defective elimination of autoimmune B cells has been demonstrated.

Keywords

Common variable immune deficiency memory B cell pathogenesis autoimmunity 

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  1. 1.Mount Sinai School of MedicineNew York CityUSA