Alpha-1-Proteinase Inhibitor and Pulmonary Haemorrhage in Systemic Vasculitis

  • Donal J. O’Donoghue
  • Mary Guickian
  • Gillian Blundell
  • Robin J. Winney
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 336)

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.

Keywords

Systemic Vasculitis Pulmonary Haemorrhage Allelic Imbalance Crescentic Glomerulonephritis Inhibitor Phenotype 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Allen, R.C., Harley, R.A., Talamo, R.C., 1974, A new method for determination of alpha-1-antityrpsin phenotypes using isoelectric focusing on polyacrylamide gel slabs. Am. J. Clin. Pathol. 62: 732–739.Google Scholar
  2. Brantly, M., Nukiwa, T., and Crystal, R.G., 1988, Molecular basis of alpha-l-antitrypsin deficiency. Am. J. Med. 84: 13–31.Google Scholar
  3. Cohen Tervaert, J.W., Goldschmeding, R., Elema, T.D., Van der Giessen, M., Huitena, M.G., Van der Hem, G.K., The, T.H., Van Den Borne, A.E.G. KR., and Kallenberg, C.G.M., 1990. Autoantibodies against myeloid lysosomal enzymes in crescentic glomerulonephritis. Kidney Int. 37: 799–806.CrossRefGoogle Scholar
  4. Ewert, B.H., Jennette, J.C., and Falk, R.J., 1992, Anti-myeloperoxidase antibodies stimulate neutrophils to damage human endothelial cells. Kidney Int. 41: 375–383.PubMedCrossRefGoogle Scholar
  5. Ewert, B.H., Jennette, J.C., Falk, R.J., 1991, The pathogenic role of antineutrophil cytoplasmic autoantibodies, Am. J. Kidney Dis. 18: 188–195.Google Scholar
  6. Falk, R.J., 1990, ANCA-associated renal disease, Kidney Int. 38: 998–1010.PubMedCrossRefGoogle Scholar
  7. Lesavre, Ph., 1991, Antineutrophil cytoplasmic autoantibodies antigen specificity, Am. J. Kid. Dis. 2: 159–163.Google Scholar
  8. Mancini, G., Vaerman, J.P., Carbonara, A.O., and Hevemans, J.F., 1963, A single radiodiffusion method for the immunological quantification of proteins. In Peeters Med. Protides of the Biological Fluids. Elsevier Amsterdam.Google Scholar
  9. Rao, N.V., Wehrer, N.G., Marshall, B.C., Gray, W.R., Gray, B.H., and Hadal, J.R., 1991, Chracterization of Proteinase 3, a neutrophil serine proteinase, H. Biol. Chem. 266: 9540–9548.Google Scholar

Copyright information

© Springer Science+Business Media New York 1993

Authors and Affiliations

  • Donal J. O’Donoghue
    • 1
  • Mary Guickian
    • 1
  • Gillian Blundell
    • 2
  • Robin J. Winney
    • 1
  1. 1.Department of Renal MedicineRoyal Infirmary of EdinburghEdinburghScotland
  2. 2.Department of Chemical PathologyRoyal Infirmary of EdinburghEdinburghScotland

Personalised recommendations