Alpha-1-proteinase inhibitor and pulmonary haemorrhage in systemic vasculitis
DJ O'Donoghue, M Guickian, G Blundell… - … and Clinical Aspects, 1993 - Springer
DJ O'Donoghue, M Guickian, G Blundell, RJ Winney
ANCA-Associated Vasculitides: Immunological and Clinical Aspects, 1993•SpringerAlpha-1-proteinase inhibitor phenotypes and levels were examined in 40 antineutrophil
cytoplasmic antibody positive cases of systemic vasculitis. An excess of PiZ and PiS alleles
were associated with the develoement of pulmonary haemorrhage and alpha-l-proteinase
inhibitor levels were lower in the subgroup with pulmonary haemorrhage. However, this
allelic imbalance and reduced alpha-1-proteinase inhibitor level was not confined to
antiproteinase 3 positive patients and did not appear to be associated with other organ …
cytoplasmic antibody positive cases of systemic vasculitis. An excess of PiZ and PiS alleles
were associated with the develoement of pulmonary haemorrhage and alpha-l-proteinase
inhibitor levels were lower in the subgroup with pulmonary haemorrhage. However, this
allelic imbalance and reduced alpha-1-proteinase inhibitor level was not confined to
antiproteinase 3 positive patients and did not appear to be associated with other organ …
Summary
Alpha-1-proteinase inhibitor phenotypes and levels were examined in 40 antineutrophil cytoplasmic antibody positive cases of systemic vasculitis. An excess of PiZ and PiS alleles were associated with the develoement of pulmonary haemorrhage and alpha-l-proteinase inhibitor levels were lower in the subgroup with pulmonary haemorrhage. However, this allelic imbalance and reduced alpha-1-proteinase inhibitor level was not confined to antiproteinase 3 positive patients and did not appear to be associated with other organ involvement or disease severity.
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