Document Detail

The autoreactivity of B cells in hereditary angioedema due to C1 inhibitor deficiency.
MedLine Citation:
PMID:  22288585     Owner:  NLM     Status:  MEDLINE    
Patients with hereditary angioedema (HAE) tend to produce autoantibodies and have a propensity to develop immunoregulatory disorders. We characterize the profile of autoantibodies in a group of HAE patients and investigate their memory B cells' phenotype and activation status. We studied the activity status phenotype, Toll-like receptor (TLR)-9 expression and total phosphotyrosine in B cells isolated from HAE patients. Additionally, the following autoantibodies were assessed in the serum of 61 HAE patients: anti-nuclear, rheumatoid factor, anti-cardiolipin, anti-tissue transglutaminase, anti-endomysial, anti-Saccharomyces cerevisiae, anti-thyroid and anti-neutrophil cytoplasmic antibodies. In 47·5% of HAE patients we detected at least one of the tested autoantibodies. Expression of CD69, CD5 and CD21 was found to be significantly higher on memory B cells from HAE patients compared to healthy controls (4·59 ± 4·41 versus 2·06 ± 1·81, P = 0·04, 8·22 ± 7·17 versus 3·65 ± 3·78, P = 0·05, 2·43 ± 0·54 versus 1·92 ± 0·41, P = 0·01, respectively). Total phosphotyrosine in B cells from HAE patients was significantly higher compared to healthy controls (4·8 ± 1·1 versus 2·7 ± 1·3, P = 0·0003). Memory B cells isolated from the HAE group contained higher amounts of TLR-9 compared to healthy controls (8·17 ± 4·1 versus 4·56 ± 1·6, P = 0·0027). Furthermore, the expression of TLR-9 in memory B cells from HAE patients with autoantibodies was significantly higher than the control group (10 ± 4·7 versus 4·56 ± 1·6, P = 0·0002) and from that in HAE patients without autoantibodies (10 ± 4·7 versus 5·8 ± 0·9, P = 0·036). HAE patients have enhanced production of autoantibodies due most probably to the increased activation of B cells, which was found to be in association with a high expression of TLR-9.
A Kessel; R Peri; R Perricone; M D Guarino; Z Vadasz; R Novak; T Haj; S Kivity; E Toubi
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical and experimental immunology     Volume:  167     ISSN:  1365-2249     ISO Abbreviation:  Clin. Exp. Immunol.     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-01-31     Completed Date:  2012-03-22     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  0057202     Medline TA:  Clin Exp Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  422-8     Citation Subset:  IM    
Copyright Information:
© 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology.
Division of Allergy and Clinical Immunology, Bnai Zion Medical Center, Technion Faculty of Medicine, Haifa, Israel.
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MeSH Terms
Autoantibodies / blood
B-Lymphocytes / classification,  immunology*
Case-Control Studies
Complement C1 Inactivator Proteins / deficiency*
Hereditary Angioedema Types I and II / etiology,  immunology*
Immunologic Memory
Lymphocyte Activation
Middle Aged
Signal Transduction / immunology
Toll-Like Receptor 9 / metabolism
Young Adult
Reg. No./Substance:
0/Autoantibodies; 0/Complement C1 Inactivator Proteins; 0/SERPING1 protein, human; 0/TLR9 protein, human; 0/Toll-Like Receptor 9

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