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©2013 UpToDate ® Print Email 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. Data from: Esnault VL, Testa A, Audrain M, et al. Alpha-1 antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis. Kidney Int 1993; 43:1329. Reproduced with permission from: American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 2003; 168:818. Official Journal of the American Thoracic Society. Copyright © 2003 American Thoracic Society.