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Kidney Int. 1993 Jun;43(6):1329-32.

Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis.

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1
Laboratoire d'Immunologie, Hotel Dieu, Nantes, France.

Abstract

Alpha 1-antitrypsin (alpha 1-AT) is the major inhibitor of proteinase 3 (PR3), the main target antigen of antineutrophil cytoplasm antibodies (ANCA) in Wegener's granulomatosis. alpha 1-AT is encoded by a polymorphic gene, with over 75 alleles, defining severely, medium and non-deficient protease inhibitor (PI) phenotypes. We describe the association of severely and medium deficient PI phenotypes with anti-PR3 positive systemic vasculitis, and postulate a pathogenetic role for alpha 1-AT deficiency and the occurrence of ANCA, with specificity for PR3 in a subgroup of patients with Wegener's granulomatosis.

PMID:
8315946
DOI:
10.1038/ki.1993.186
[Indexed for MEDLINE]
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