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
(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
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
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
but those who do develop vasculitis have a worse prognosis.
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Vasculitis (Wegener's PAN, etc.)