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Homozygosity for the IgG2 subclass allotype G2M(n) protects against severe infection in hereditary C2 deficiency

Jönsson, Göran LU ; Oxelius, Vivi-Anne LU ; Truedsson, Lennart LU ; Braconier, Jean Henrik LU ; Sturfelt, Gunnar LU and Sjöholm, Anders LU (2006) In Journal of Immunology 177(1). p.722-728
Abstract
Homozygous C2 deficiency (C2D) is the most common deficiency of the classical complement pathway in Western countries. It is mostly found in patients with autoimmune disease or susceptibility to bacterial infections and in healthy persons. We wished to assess to what extent other immunological factors might explain differences of susceptibility to infections in C2D. For this reason, 44 Swedish patients with C2D were stratified with regard to the severity of documented infections. Investigations of IgG subclass levels, IgG subclass-specific GM allotypes, concentrations of factor B, properdin, and factor H, and polymorphisms of mannan-binding lectin and the Fe receptors Fc gamma RIIa and Fc gamma RIIIb were performed. Homozygosity for the... (More)
Homozygous C2 deficiency (C2D) is the most common deficiency of the classical complement pathway in Western countries. It is mostly found in patients with autoimmune disease or susceptibility to bacterial infections and in healthy persons. We wished to assess to what extent other immunological factors might explain differences of susceptibility to infections in C2D. For this reason, 44 Swedish patients with C2D were stratified with regard to the severity of documented infections. Investigations of IgG subclass levels, IgG subclass-specific GM allotypes, concentrations of factor B, properdin, and factor H, and polymorphisms of mannan-binding lectin and the Fe receptors Fc gamma RIIa and Fc gamma RIIIb were performed. Homozygosity for the G2M*n allele, which is known to promote Ab responses to polysaccharide Ags, was strongly associated with the absence of severe infections (P < 0.001) in the patients, suggesting a major protective role. The combination of mannan (or mannose)-binding lectin and C2 deficiency was found to be a minor susceptibility factor for invasive infection (p = 0.03). Low concentrations of IgG2 and factor B might sometimes contribute to susceptibility to infection. Other factors investigated did not appear to be important. In conclusion, the findings indicated that efficient Ab responses to polysaccharides are protective against severe infection in C2D. Implications with regard to vaccinations should be considered. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Journal of Immunology
volume
177
issue
1
pages
722 - 728
publisher
American Association of Immunologists
external identifiers
  • wos:000238471400084
  • pmid:16785571
  • scopus:33745307266
ISSN
1550-6606
language
English
LU publication?
yes
id
7dcffa95-8cf5-40da-9c61-b6274b849b10 (old id 404884)
alternative location
http://www.jimmunol.org/cgi/content/abstract/177/1/722
date added to LUP
2007-10-07 11:56:43
date last changed
2019-02-20 08:24:10
@article{7dcffa95-8cf5-40da-9c61-b6274b849b10,
  abstract     = {Homozygous C2 deficiency (C2D) is the most common deficiency of the classical complement pathway in Western countries. It is mostly found in patients with autoimmune disease or susceptibility to bacterial infections and in healthy persons. We wished to assess to what extent other immunological factors might explain differences of susceptibility to infections in C2D. For this reason, 44 Swedish patients with C2D were stratified with regard to the severity of documented infections. Investigations of IgG subclass levels, IgG subclass-specific GM allotypes, concentrations of factor B, properdin, and factor H, and polymorphisms of mannan-binding lectin and the Fe receptors Fc gamma RIIa and Fc gamma RIIIb were performed. Homozygosity for the G2M*n allele, which is known to promote Ab responses to polysaccharide Ags, was strongly associated with the absence of severe infections (P &lt; 0.001) in the patients, suggesting a major protective role. The combination of mannan (or mannose)-binding lectin and C2 deficiency was found to be a minor susceptibility factor for invasive infection (p = 0.03). Low concentrations of IgG2 and factor B might sometimes contribute to susceptibility to infection. Other factors investigated did not appear to be important. In conclusion, the findings indicated that efficient Ab responses to polysaccharides are protective against severe infection in C2D. Implications with regard to vaccinations should be considered.},
  author       = {Jönsson, Göran and Oxelius, Vivi-Anne and Truedsson, Lennart and Braconier, Jean Henrik and Sturfelt, Gunnar and Sjöholm, Anders},
  issn         = {1550-6606},
  language     = {eng},
  number       = {1},
  pages        = {722--728},
  publisher    = {American Association of Immunologists},
  series       = {Journal of Immunology},
  title        = {Homozygosity for the IgG2 subclass allotype G2M(n) protects against severe infection in hereditary C2 deficiency},
  volume       = {177},
  year         = {2006},
}