The role of α1-antitrypsin deficiency in the pathogenesis of antineutrophil cytoplasmic antibodies associated systemic necrotizing vasculitides

Abstract EN

Alpha1-antitrypsin (D,1AT) is the most abundant circulating protease inhibitor (Pi) in human plasma.

It has central function in controlling tissue degradation by inhibiting a large number of proteases including neutrophil elastase and proteinase 3 (PR3).

PR3, the Wegner's autoantigen, has been suggested to be involved in the pathogenesis of smallvessel systemic vasculitides.

α1 AT deficiency (PiZ) is frequent in Caucasian populations, and its homozygous state (PiZZ) is known to predispose to lung emphysema and chronic liver disease.

A strong correlation between heterozygous (PiZ) and homozygous (PiZZ) α1 AT deficiency and anti-neutrophil cytoplasmic autoantibodies (ANCA) associated systemic nocrotizing vasculitides has recently been reported in various populations.

In this review the pathogenesis of small-vessel vasculitides is outlined, focusing on the role of α1 AT deficiency.

α1 AT has been suggested to have a crucial role as a protective protein in ANCA-associated vasculitic syndromes.