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Complement deficiency and disease: An update.

Sjöholm, Anders LU ; Jönsson, Göran LU ; Braconier, Jean Henrik LU ; Sturfelt, Gunnar LU and Truedsson, Lennart LU (2006) In Molecular Immunology 43(1-2). p.78-85
Abstract
Complement deficiencies are probably vastly under-diagnosed within clinical medicine. Judging from a Swedish study of C2 deficiency, a deficiency with an estimated prevalence of about 1/20,000 in Western countries, less than 10% of the deficiencies of the classical and alternative pathways and the late complement components are identified in Sweden. C1 inhibitor deficiency and deficiencies of MBL and MASP-2 were not included in the assessment. The introduction of new screening methods should facilitate detection of complement deficiencies in clinical practice. In our study of C2 deficiency (n = 40), 57% of the patients had a history of invasive infection with encapsulated bacteria, mainly Streptococcus pneumoniae. This emphasizes the... (More)
Complement deficiencies are probably vastly under-diagnosed within clinical medicine. Judging from a Swedish study of C2 deficiency, a deficiency with an estimated prevalence of about 1/20,000 in Western countries, less than 10% of the deficiencies of the classical and alternative pathways and the late complement components are identified in Sweden. C1 inhibitor deficiency and deficiencies of MBL and MASP-2 were not included in the assessment. The introduction of new screening methods should facilitate detection of complement deficiencies in clinical practice. In our study of C2 deficiency (n = 40), 57% of the patients had a history of invasive infection with encapsulated bacteria, mainly Streptococcus pneumoniae. This emphasizes the importance of the classical and/or the lectin pathway in defence against severe infection. Rheumatological disease, mainly systemic lupus erythematosus was present in 43% of the patients. In addition, a significant association was found between C2 deficiency and atherosclerosis. Complement-dependent disease mechanisms are discussed together with the potential importance of non-complement genes for disease expression in complement deficiencies. Analysis of larger patient groups is required in order to establish guidelines for investigation and treatment of patients with complement deficiency. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Atherosclerosis, Septicemia, Systemic lupus erythematosus, Complement, Immunodeficiency
in
Molecular Immunology
volume
43
issue
1-2
pages
78 - 85
publisher
Pergamon
external identifiers
  • scopus:22544485955
  • wos:000232021600009
ISSN
1872-9142
DOI
10.1016/j.molimm.2005.06.025
language
English
LU publication?
yes
id
84b49903-77fc-4f01-bfb7-d0b33a2fd731 (old id 1136224)
date added to LUP
2007-07-04 11:05:37
date last changed
2019-03-10 04:06:54
@article{84b49903-77fc-4f01-bfb7-d0b33a2fd731,
  abstract     = {Complement deficiencies are probably vastly under-diagnosed within clinical medicine. Judging from a Swedish study of C2 deficiency, a deficiency with an estimated prevalence of about 1/20,000 in Western countries, less than 10% of the deficiencies of the classical and alternative pathways and the late complement components are identified in Sweden. C1 inhibitor deficiency and deficiencies of MBL and MASP-2 were not included in the assessment. The introduction of new screening methods should facilitate detection of complement deficiencies in clinical practice. In our study of C2 deficiency (n = 40), 57% of the patients had a history of invasive infection with encapsulated bacteria, mainly Streptococcus pneumoniae. This emphasizes the importance of the classical and/or the lectin pathway in defence against severe infection. Rheumatological disease, mainly systemic lupus erythematosus was present in 43% of the patients. In addition, a significant association was found between C2 deficiency and atherosclerosis. Complement-dependent disease mechanisms are discussed together with the potential importance of non-complement genes for disease expression in complement deficiencies. Analysis of larger patient groups is required in order to establish guidelines for investigation and treatment of patients with complement deficiency.},
  author       = {Sjöholm, Anders and Jönsson, Göran and Braconier, Jean Henrik and Sturfelt, Gunnar and Truedsson, Lennart},
  issn         = {1872-9142},
  keyword      = {Atherosclerosis,Septicemia,Systemic lupus erythematosus,Complement,Immunodeficiency},
  language     = {eng},
  number       = {1-2},
  pages        = {78--85},
  publisher    = {Pergamon},
  series       = {Molecular Immunology},
  title        = {Complement deficiency and disease: An update.},
  url          = {http://dx.doi.org/10.1016/j.molimm.2005.06.025},
  volume       = {43},
  year         = {2006},
}