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Journal of Clinical Immunology

Published in:

01-05-2008

Autoimmune Manifestations in Common Variable Immunodeficiency

Author: C. Cunningham-Rundles

Published in: Journal of Clinical Immunology | Special Issue 1/2008

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.

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Title: Autoimmune Manifestations in Common Variable Immunodeficiency
Author: C. Cunningham-Rundles
Publication date: 01-05-2008
Publisher: Springer US
Published in: Journal of Clinical Immunology / Issue Special Issue 1/2008
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-008-9182-7

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