Article
ANCA-associated vasculitis is linked to carriage of the Z allele of α₁ antitrypsin and its polymers.
Annals of the rheumatic diseases - 1 Oct 2011
Morris H, Morgan M D, Wood A M, Smith S W, Ekeowa U I, Herrmann K, Holle J U, Guillevin L, Lomas D A, Perez J, Pusey C D, Salama A D, Stockley R, Wieczorek S, McKnight A J, Maxwell A P, Miranda E, Williams J, Savage C O, Harper L
Abstract excerpt
BACKGROUND: Small studies have linked α1 antitrypsin (α1AT) deficiency to patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). OBJECTIVE: To test the validity and the mechanism of this association between α1AT and AAV. METHODS: The distribution of α1AT deficiency alleles Z and S was compared between 856 White Europeans with AAV and 1505 geographic and ethnically matched healthy...
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