C3 and C4 allotypes in anti-neutrophil cytoplasmic autoantibody (ANCA)- positive vasculitis
(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
- Persson, U. LU ; Truedsson, L. LU ; Westman, K. W.A. LU and Segelmark, Mårten LU
- organization
- publishing date
- 1999
- 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
- 2024-01-02 11:13:51
@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<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}}, keywords = {{ANCA; Complement proteinase 3; Vasculitis; Wegener's granulomatosis}}, 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}}, }