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Saudi Journal of Kidney Diseases and Transplantation
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Year : 1999  |  Volume : 10  |  Issue : 1  |  Page : 41-53
The role of α1 -antitrypsin Deficiency in the Pathogenesis of Antineutrophil Cytoplasmic Antibodies Associated Systemic Necrotizing Vasculitides


Department of Medicine, University Hospital, Al-Arab Medical University, Benghazi, Libya

Correspondence Address:
Abdul-Nasser Alzouki
Department of Medicine, University Hospital, Al-Arab Medical University, P.O. Box 7224, Alberka Post Office, Benghazi
Libya
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PMID: 18212413

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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.


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