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C3 and C4 allotypes in anti-neutrophil cytoplasmic autoantibody (ANCA)- positive vasculitis

Persson, U. ; Truedsson, L. LU ; Westman, K. W.A. LU and Segelmark, Mårten LU (1999) In Clinical and Experimental Immunology 116(2). p.379-382
Abstract

In ANCA-associated small vessel vasculitis few genetic factors have proven to be of importance for disease susceptibility, an exception being deficiency of α1-anti-trypsin, the main inhibitor of proteinase 3 (PR3). Alerted by our finding that myeloperoxidase has affinity for C3, and the finding of an increased frequency of the C3F allele in systemic vasculitis in a British cohort, we examined polymorphism of C3 and C4 in patients with ANCA+ small vessel vasculitis. After identification of all patients at our department with a positive ANCA test during the period 1991-95 and a diagnosis of small vessel vasculitis, blood samples were collected after informed consent. The 67 included patients were grouped according to... (More)

In ANCA-associated small vessel vasculitis few genetic factors have proven to be of importance for disease susceptibility, an exception being deficiency of α1-anti-trypsin, the main inhibitor of proteinase 3 (PR3). Alerted by our finding that myeloperoxidase has affinity for C3, and the finding of an increased frequency of the C3F allele in systemic vasculitis in a British cohort, we examined polymorphism of C3 and C4 in patients with ANCA+ small vessel vasculitis. After identification of all patients at our department with a positive ANCA test during the period 1991-95 and a diagnosis of small vessel vasculitis, blood samples were collected after informed consent. The 67 included patients were grouped according to ANCA serology and disease phenotype using the Chapel Hill nomenclature. The gene frequency of C3F was found to be increased (0-32) compared with controls (0.20; P<0.05) in the PR3-ANCA+ subgroup. The frequency of C4A3 was increased in the group as a whole, but no increase of C4 null alleles was seen. The findings imply a role for the complement system in the pathogenesis of ANCA-associated small vessel vasculitis.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
ANCA, Complement proteinase 3, Vasculitis, Wegener's granulomatosis
in
Clinical and Experimental Immunology
volume
116
issue
2
pages
379 - 382
publisher
British Society for Immunology
external identifiers
  • scopus:0032918886
  • pmid:10337034
ISSN
0009-9104
DOI
10.1046/j.1365-2249.1999.00889.x
language
English
LU publication?
yes
id
87600817-3e05-4379-be98-eb39a23121c4
date added to LUP
2020-05-20 16:58:18
date last changed
2020-05-21 01:57:57
@article{87600817-3e05-4379-be98-eb39a23121c4,
  abstract     = {<p>In ANCA-associated small vessel vasculitis few genetic factors have proven to be of importance for disease susceptibility, an exception being deficiency of α<sub>1</sub>-anti-trypsin, the main inhibitor of proteinase 3 (PR3). Alerted by our finding that myeloperoxidase has affinity for C3, and the finding of an increased frequency of the C3F allele in systemic vasculitis in a British cohort, we examined polymorphism of C3 and C4 in patients with ANCA<sup>+</sup> small vessel vasculitis. After identification of all patients at our department with a positive ANCA test during the period 1991-95 and a diagnosis of small vessel vasculitis, blood samples were collected after informed consent. The 67 included patients were grouped according to ANCA serology and disease phenotype using the Chapel Hill nomenclature. The gene frequency of C3F was found to be increased (0-32) compared with controls (0.20; P&lt;0.05) in the PR3-ANCA<sup>+</sup> subgroup. The frequency of C4A3 was increased in the group as a whole, but no increase of C4 null alleles was seen. The findings imply a role for the complement system in the pathogenesis of ANCA-associated small vessel vasculitis.</p>},
  author       = {Persson, U. and Truedsson, L. and Westman, K. W.A. and Segelmark, Mårten},
  issn         = {0009-9104},
  language     = {eng},
  number       = {2},
  pages        = {379--382},
  publisher    = {British Society for Immunology},
  series       = {Clinical and Experimental Immunology},
  title        = {C3 and C4 allotypes in anti-neutrophil cytoplasmic autoantibody (ANCA)- positive vasculitis},
  url          = {http://dx.doi.org/10.1046/j.1365-2249.1999.00889.x},
  doi          = {10.1046/j.1365-2249.1999.00889.x},
  volume       = {116},
  year         = {1999},
}