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The PiZ gene of α1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis

Segelmark, Mårten LU orcid ; Elzouki, Abdul Nasser LU ; Wieslander, Jörgen LU and Eriksson, Sten LU (1995) In Kidney International 48(3). p.844-850
Abstract

We have previously demonstrated that a strong correlation exists between systemic vasculitis with proteinase 3-anti-neutrophil cytoplasm antibodies (PR3-ANCA) and heterozygosity for α1-antitrypsin (α1-AT) deficiency, PiZ. In the present study we characterized the PiZ-positive subgroup by laboratory findings, clinical features and outcome. The series studied consisted of 18 PiZ-positive and 81 PiZ-negative PR3-ANCA patients, comparable in sex ratio, age, C-reactive protein concentrations and renal function at-diagnosis, and treatment. PiZ-positive patients had a more disseminated disease as reflected by the number of affected organs (P < 0.01). We found no group differences in relapse tendency. Overall mortality... (More)

We have previously demonstrated that a strong correlation exists between systemic vasculitis with proteinase 3-anti-neutrophil cytoplasm antibodies (PR3-ANCA) and heterozygosity for α1-antitrypsin (α1-AT) deficiency, PiZ. In the present study we characterized the PiZ-positive subgroup by laboratory findings, clinical features and outcome. The series studied consisted of 18 PiZ-positive and 81 PiZ-negative PR3-ANCA patients, comparable in sex ratio, age, C-reactive protein concentrations and renal function at-diagnosis, and treatment. PiZ-positive patients had a more disseminated disease as reflected by the number of affected organs (P < 0.01). We found no group differences in relapse tendency. Overall mortality was 39% (7 of 18) in the PiZ-positive and 16% (13 of 81) in the non-piZ group (P = 0.048). When survival analysis was restricted to 66 patients included in the study at disease onset, the group difference was significant (P = 0.016). The results suggest that the subnormal response of plasma α1-AT seen in PiZ-heterozygotes enhances the risk of dissemination of the vasculitic process and the risk of a fatal outcome. We consider α1-AT phenotyping to be justified in cases of PR3-ANCA-positive vasculitis. Treatments decreasing plasma α1-AT (such as plasmapheresis without plasma replacement) may be deleterious.

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author
; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Kidney International
volume
48
issue
3
pages
844 - 850
publisher
Nature Publishing Group
external identifiers
  • pmid:7474674
  • scopus:0029144094
ISSN
0085-2538
DOI
10.1038/ki.1995.360
language
English
LU publication?
yes
id
b8c7a1ba-ba53-4455-b7f9-dabf055b1e6f
date added to LUP
2020-05-20 16:39:24
date last changed
2025-10-14 10:05:30
@article{b8c7a1ba-ba53-4455-b7f9-dabf055b1e6f,
  abstract     = {{<p>We have previously demonstrated that a strong correlation exists between systemic vasculitis with proteinase 3-anti-neutrophil cytoplasm antibodies (PR3-ANCA) and heterozygosity for α<sub>1</sub>-antitrypsin (α<sub>1</sub>-AT) deficiency, PiZ. In the present study we characterized the PiZ-positive subgroup by laboratory findings, clinical features and outcome. The series studied consisted of 18 PiZ-positive and 81 PiZ-negative PR3-ANCA patients, comparable in sex ratio, age, C-reactive protein concentrations and renal function at-diagnosis, and treatment. PiZ-positive patients had a more disseminated disease as reflected by the number of affected organs (P &lt; 0.01). We found no group differences in relapse tendency. Overall mortality was 39% (7 of 18) in the PiZ-positive and 16% (13 of 81) in the non-piZ group (P = 0.048). When survival analysis was restricted to 66 patients included in the study at disease onset, the group difference was significant (P = 0.016). The results suggest that the subnormal response of plasma α<sub>1</sub>-AT seen in PiZ-heterozygotes enhances the risk of dissemination of the vasculitic process and the risk of a fatal outcome. We consider α<sub>1</sub>-AT phenotyping to be justified in cases of PR3-ANCA-positive vasculitis. Treatments decreasing plasma α<sub>1</sub>-AT (such as plasmapheresis without plasma replacement) may be deleterious.</p>}},
  author       = {{Segelmark, Mårten and Elzouki, Abdul Nasser and Wieslander, Jörgen and Eriksson, Sten}},
  issn         = {{0085-2538}},
  language     = {{eng}},
  number       = {{3}},
  pages        = {{844--850}},
  publisher    = {{Nature Publishing Group}},
  series       = {{Kidney International}},
  title        = {{The PiZ gene of α<sub>1</sub>-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis}},
  url          = {{http://dx.doi.org/10.1038/ki.1995.360}},
  doi          = {{10.1038/ki.1995.360}},
  volume       = {{48}},
  year         = {{1995}},
}