Segelmark M, Elzouki A-N, Wieslander J, Eriksson S
The PiZ gene of alpha-1 antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis

Kidney Int (Sep) 48:844-850 1995

Vasculitis, particularly Wegener's, is often associated with antibodies to proteinase 3. The natural serum inhibitor of proteinase 3 is a1-antitrypsin. Vasculitis patients with activating antibodies to proteinase 3 might have more inflammation if they lacked the proteinase inhibitor. In this study 105 PR3-ANCA-positive patients were studied to determine the effect of heterozygosity for PiZ, an a1-antitrypsin allele with decreased activity. Eighteen patients were PiZ positive. They were similar in disease presentation to those with normal a1-antitrypsin activity. However, PiZ patients had increased overall mortality: 39% (7/18) vs 16% (13/81) in controls (p=0.048). For those patients with elevated serum creatinine at presentation, PiZ heterozygosity resulted in a poorer outcome (decreased patient or renal survival). It appears that PiZ heterozygosity does not predispose to vasculitis, but those who do develop vasculitis have a worse prognosis. (Paller)

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Proteinuria/Hematuria : Vasculitis (Wegener's PAN, etc.)