Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura
Abstract Henoch–Schönlein purpura (HSP) is a vasculitis of unknown aetiology, possibly involving immune complexes. The complement system is essential for the clearance of immune complexes. Our aim was to explore the hypothesis that patients with HSP have abnormal complements, contributing to the dev...
Published in: | Scandinavian Journal of Immunology |
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crwiley:10.1111/j.1365-3083.2005.01533.x 2024-06-02T08:09:19+00:00 Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura Stefansson Thors, V. Kolka, R. Sigurdardottir, S. L. Edvardsson, V. O. Arason, G. Haraldsson, A. 2005 http://dx.doi.org/10.1111/j.1365-3083.2005.01533.x https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1365-3083.2005.01533.x https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1365-3083.2005.01533.x en eng Wiley http://onlinelibrary.wiley.com/termsAndConditions#vor Scandinavian Journal of Immunology volume 61, issue 3, page 274-278 ISSN 0300-9475 1365-3083 journal-article 2005 crwiley https://doi.org/10.1111/j.1365-3083.2005.01533.x 2024-05-03T12:00:44Z Abstract Henoch–Schönlein purpura (HSP) is a vasculitis of unknown aetiology, possibly involving immune complexes. The complement system is essential for the clearance of immune complexes. Our aim was to explore the hypothesis that patients with HSP have abnormal complements, contributing to the development of the disease. The study included 56 patients diagnosed with HSP at the Children's Hospital, Iceland between 1984 and 2000, and 98 blood donors as controls. Serum levels of immunoglobulin A, C4A, C4B and mannan‐binding lectin were measured and compared between the two groups. C4 null alleles were significantly more common in HSP patients than in controls ( P = 0.018) and were carried by 66.1% of the patients compared with 41.2% of the controls. This difference was due to an increased frequency of C4B*Q0 allele in the HSP group (0.25 versus 0.11 in the control group; P = 0.002). The fact that the majority of our patients carried a C4 null allele indicates that children with C4 deficiencies may have an increased risk of developing HSP. This may reflect inadequate complement activity and possibly present an opportunity to identify patients at risk of developing serious morbidity associated with HSP. Article in Journal/Newspaper Iceland Wiley Online Library Scandinavian Journal of Immunology 61 3 274 278 |
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Wiley Online Library |
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language |
English |
description |
Abstract Henoch–Schönlein purpura (HSP) is a vasculitis of unknown aetiology, possibly involving immune complexes. The complement system is essential for the clearance of immune complexes. Our aim was to explore the hypothesis that patients with HSP have abnormal complements, contributing to the development of the disease. The study included 56 patients diagnosed with HSP at the Children's Hospital, Iceland between 1984 and 2000, and 98 blood donors as controls. Serum levels of immunoglobulin A, C4A, C4B and mannan‐binding lectin were measured and compared between the two groups. C4 null alleles were significantly more common in HSP patients than in controls ( P = 0.018) and were carried by 66.1% of the patients compared with 41.2% of the controls. This difference was due to an increased frequency of C4B*Q0 allele in the HSP group (0.25 versus 0.11 in the control group; P = 0.002). The fact that the majority of our patients carried a C4 null allele indicates that children with C4 deficiencies may have an increased risk of developing HSP. This may reflect inadequate complement activity and possibly present an opportunity to identify patients at risk of developing serious morbidity associated with HSP. |
format |
Article in Journal/Newspaper |
author |
Stefansson Thors, V. Kolka, R. Sigurdardottir, S. L. Edvardsson, V. O. Arason, G. Haraldsson, A. |
spellingShingle |
Stefansson Thors, V. Kolka, R. Sigurdardottir, S. L. Edvardsson, V. O. Arason, G. Haraldsson, A. Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura |
author_facet |
Stefansson Thors, V. Kolka, R. Sigurdardottir, S. L. Edvardsson, V. O. Arason, G. Haraldsson, A. |
author_sort |
Stefansson Thors, V. |
title |
Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura |
title_short |
Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura |
title_full |
Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura |
title_fullStr |
Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura |
title_full_unstemmed |
Increased Frequency of C4B*Q0 Alleles in Patients with Henoch–Schönlein Purpura |
title_sort |
increased frequency of c4b*q0 alleles in patients with henoch–schönlein purpura |
publisher |
Wiley |
publishDate |
2005 |
url |
http://dx.doi.org/10.1111/j.1365-3083.2005.01533.x https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1111%2Fj.1365-3083.2005.01533.x https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1365-3083.2005.01533.x |
genre |
Iceland |
genre_facet |
Iceland |
op_source |
Scandinavian Journal of Immunology volume 61, issue 3, page 274-278 ISSN 0300-9475 1365-3083 |
op_rights |
http://onlinelibrary.wiley.com/termsAndConditions#vor |
op_doi |
https://doi.org/10.1111/j.1365-3083.2005.01533.x |
container_title |
Scandinavian Journal of Immunology |
container_volume |
61 |
container_issue |
3 |
container_start_page |
274 |
op_container_end_page |
278 |
_version_ |
1800755019019976704 |