Journal article

ALPHA(1)-ANTITRYPSIN DEFICIENCY AND ANTIPROTEINASE 3 ANTIBODIES IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED SYSTEMIC VASCULITIS

JA SAVIGE, L CHANG, L COOK, J BURDON, M DASKALAKIS, J DOERY

CLINICAL AND EXPERIMENTAL IMMUNOLOGY | BLACKWELL SCIENCE LTD | Published : 1995

Abstract

alpha 1-antitrypsin (alpha 1-AT) is a naturally occurring inhibitor of proteinase 3 (PR3) and elastase, two of the target antigens of anti-neutrophil cytoplasmic antibodies (ANCA). An increased incidence of alpha 1-AT phenotypes associated with dysfunctional alpha 1-AT or low serum levels has been reported in patients with anti-PR3 antibodies. We have studied the relationship between ANCA, and phenotypes and serum levels of alpha 1-AT. Phenotypes usually associated with a moderate or severe reduction in alpha 1-AT serum levels or in dysfunctional activity were found more often in individuals with anti-PR3 antibodies than in the general population: four of the 31 patients (13%) with anti-PR3 ..

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