Alpha-1 antitrypsin phenotypes in anti-PR3 (C-ANCA) positive vasculitic disorders
Alpha-1 antitrypsin phenotypes in anti-PR3 (C-ANCA) positive vasculitic disorders
France
Sweden
Austria
Australia
UK
Denmark
Number of patients
37
105
32
31
99
44
PI*ZZ
3
1
2
1
1
0
Z allele frequency
17.6
9
10.9
6.5
5.6
9.1
Z allele in controls
1.9
2.4
1.4
0.9
1.8
2.4
anti-PR3: antibody to proteinase 3; C-ANCA: cytoplasmic staining pattern of antineutrophil cytoplasmic antibodies; PI: protease inhibitor; PI*ZZ: homozygosity of protease inhibitor allele (Z) that is most commonly associated with alpha-1 antitrypsin deficiency.