Article
Alpha 1-antitrypsin phenotypes in patients with anti-neutrophil cytoplasmic antibody-positive vasculitis.
Clinical science (London, England : 1979) - 1 Dec 1994
Lhotta K, Vogel W, Meisl T, Buxbaum M, Neyer U, Sandholzer C, König P
Abstract excerpt
1. The genetic background of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis remains largely unknown. Recently a very high prevalence of medium and severe deficiency of alpha 1-antitrypsin was described in a small group of patients with Wegener's granulomatosis and c-ANCA. c-ANCAs are autoantibodies against proteinase 3, and alpha 1-antitrypsin is the main inhibitor of this enzyme. 2....
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