The PiZ gene of α1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis
(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.
(Less)
- author
- Segelmark, Mårten LU ; Elzouki, Abdul Nasser LU ; Wieslander, Jörgen LU and Eriksson, Sten LU
- organization
- publishing date
- 1995
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Kidney International
- volume
- 48
- issue
- 3
- pages
- 844 - 850
- publisher
- Nature Publishing Group
- external identifiers
-
- scopus:0029144094
- pmid:7474674
- 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
- 2024-01-02 11:12:16
@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 < 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}}, }