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.

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خرید پکیج

Alpha-1 antitrypsin phenotypes in anti-PR3 (C-ANCA) positive vasculitic disorders