Skip to main content
Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 1993 Feb;52(2):115–120. doi: 10.1136/ard.52.2.115

Occurrence of autoantibodies to human leucocyte elastase in Wegener's granulomatosis and other inflammatory disorders.

J W Tervaert 1, L Mulder 1, C Stegeman 1, J Elema 1, M Huitema 1, H The 1, C Kallenberg 1
PMCID: PMC1004989  PMID: 8383482

Abstract

Antineutrophil cytoplasmic antibodies (ANCAs) constitute a new class of autoantibodies that seem to recognise myeloid lysosomal enzymes. The occurrence of ANCAs with specificity for human leucocyte elastase (HLE) was assessed in serum samples that were routinely submitted for ANCA determination. During a study period of more than six years anti-HLE was found in only six out of 1102 serum samples that produced a perinuclear or an atypical cytoplasmic staining pattern on ethanol fixed granulocytes. These six serum samples were from four patients with a clinical diagnosis of Wegener's granulomatosis but without a definite histological diagnosis, one patient with systemic vasculitis, and one patient with Cogan's syndrome. To further evaluate the prevalence of anti-HLE we tested 315 serum samples from patients with different forms of vasculitis and related disorders. Anti-HLE was detected in two patients only. Thus autoantibodies to HLE are rarely found in serum samples from patients with vasculitic or related disorders.

Full text

PDF

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Boudes P. Purely granulomatous Wegener's granulomatosis: a new concept for an old disease. Semin Arthritis Rheum. 1990 Jun;19(6):365–370. doi: 10.1016/0049-0172(90)90073-o. [DOI] [PubMed] [Google Scholar]
  2. Campanelli D., Melchior M., Fu Y., Nakata M., Shuman H., Nathan C., Gabay J. E. Cloning of cDNA for proteinase 3: a serine protease, antibiotic, and autoantigen from human neutrophils. J Exp Med. 1990 Dec 1;172(6):1709–1715. doi: 10.1084/jem.172.6.1709. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Duerr R. H., Targan S. R., Landers C. J., LaRusso N. F., Lindsay K. L., Wiesner R. H., Shanahan F. Neutrophil cytoplasmic antibodies: a link between primary sclerosing cholangitis and ulcerative colitis. Gastroenterology. 1991 May;100(5 Pt 1):1385–1391. [PubMed] [Google Scholar]
  4. Falk R. J., Jennette J. C. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med. 1988 Jun 23;318(25):1651–1657. doi: 10.1056/NEJM198806233182504. [DOI] [PubMed] [Google Scholar]
  5. Fienberg R. The protracted superficial phenomenon in pathergic (Wegener's) granulomatosis. Hum Pathol. 1981 May;12(5):458–467. doi: 10.1016/s0046-8177(81)80027-5. [DOI] [PubMed] [Google Scholar]
  6. Gallicchio M. C., Savige J. A. Detection of anti-myeloperoxidase and anti-elastase antibodies in vasculitides and infections. Clin Exp Immunol. 1991 May;84(2):232–237. doi: 10.1111/j.1365-2249.1991.tb08154.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Goldschmeding R., van der Schoot C. E., ten Bokkel Huinink D., Hack C. E., van den Ende M. E., Kallenberg C. G., von dem Borne A. E. Wegener's granulomatosis autoantibodies identify a novel diisopropylfluorophosphate-binding protein in the lysosomes of normal human neutrophils. J Clin Invest. 1989 Nov;84(5):1577–1587. doi: 10.1172/JCI114335. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Hoffman G. S., Sechler J. M., Gallin J. I., Shelhamer J. H., Suffredini A., Ognibene F. P., Baltaro R. J., Fleisher T. A., Leavitt R. Y., Travis W. D. Bronchoalveolar lavage analysis in Wegener's granulomatosis. A method to study disease pathogenesis. Am Rev Respir Dis. 1991 Feb;143(2):401–407. doi: 10.1164/ajrccm/143.2.401. [DOI] [PubMed] [Google Scholar]
  9. Jennette J. C., Hoidal J. R., Falk R. J. Specificity of anti-neutrophil cytoplasmic autoantibodies for proteinase 3. Blood. 1990 Jun 1;75(11):2263–2264. [PubMed] [Google Scholar]
  10. Kallenberg C. G., Tervaert J. W., van der Woude F. J., Goldschmeding R., von dem Borne A. E., Weening J. J. Autoimmunity to lysosomal enzymes: new clues to vasculitis and glomerulonephritis? Immunol Today. 1991 Feb;12(2):61–64. doi: 10.1016/0167-5699(91)90159-q. [DOI] [PubMed] [Google Scholar]
  11. Leavitt R. Y., Fauci A. S., Bloch D. A., Michel B. A., Hunder G. G., Arend W. P., Calabrese L. H., Fries J. F., Lie J. T., Lightfoot R. W., Jr The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990 Aug;33(8):1101–1107. doi: 10.1002/art.1780330807. [DOI] [PubMed] [Google Scholar]
  12. Lee S. S., Adu D., Thompson R. A. Anti-myeloperoxidase antibodies in systemic vasculitis. Clin Exp Immunol. 1990 Jan;79(1):41–46. doi: 10.1111/j.1365-2249.1990.tb05124.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Lesavre P. Antineutrophil cytoplasmic autoantibodies antigen specificity. Am J Kidney Dis. 1991 Aug;18(2):159–163. doi: 10.1016/s0272-6386(12)80873-0. [DOI] [PubMed] [Google Scholar]
  14. Lüdemann J., Utecht B., Gross W. L. Anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis recognize an elastinolytic enzyme. J Exp Med. 1990 Jan 1;171(1):357–362. doi: 10.1084/jem.171.1.357. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Mark E. J., Matsubara O., Tan-Liu N. S., Fienberg R. The pulmonary biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: a study based on 35 open lung biopsies. Hum Pathol. 1988 Sep;19(9):1065–1071. doi: 10.1016/s0046-8177(88)80088-1. [DOI] [PubMed] [Google Scholar]
  16. Niles J. L., McCluskey R. T., Ahmad M. F., Arnaout M. A. Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase. Blood. 1989 Nov 1;74(6):1888–1893. [PubMed] [Google Scholar]
  17. Nässberger L., Jonsson H., Sjöholm A. G., Sturfelt G., Heubner A. Circulating anti-elastase in systemic lupus erythematosus. Lancet. 1989 Mar 4;1(8636):509–509. doi: 10.1016/s0140-6736(89)91420-7. [DOI] [PubMed] [Google Scholar]
  18. Nässberger L., Sjöholm A. G., Jonsson H., Sturfelt G., Akesson A. Autoantibodies against neutrophil cytoplasm components in systemic lupus erythematosus and in hydralazine-induced lupus. Clin Exp Immunol. 1990 Sep;81(3):380–383. doi: 10.1111/j.1365-2249.1990.tb05342.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Nölle B., Specks U., Lüdemann J., Rohrbach M. S., DeRemee R. A., Gross W. L. Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med. 1989 Jul 1;111(1):28–40. doi: 10.7326/0003-4819-111-1-28. [DOI] [PubMed] [Google Scholar]
  20. Ramírez G., Khamashta M. A., Hughes G. R. The ANCA test: its clinical relevance. Ann Rheum Dis. 1990 Oct;49(10):741–742. doi: 10.1136/ard.49.10.741. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Rasmussen N., Wiik A. Indirect immunofluorescence examination for IgG-ANCA in sera submitted for the 1st international workshop on ANCA, 1988. APMIS Suppl. 1989;6:16–20. [PubMed] [Google Scholar]
  22. Shah I. A., Holstege A., Riede U. N. Bioptic diagnosis of Wegener's granulomatosis in the absence of vasculitis and granulomas. Pathol Res Pract. 1984 Mar;178(4):407–415. doi: 10.1016/S0344-0338(84)80042-4. [DOI] [PubMed] [Google Scholar]
  23. Tervaert J. W., Goldschmeding R., Elema J. D., Limburg P. C., van der Giessen M., Huitema M. G., Koolen M. I., Hené R. J., The T. H., van der Hem G. K. Association of autoantibodies to myeloperoxidase with different forms of vasculitis. Arthritis Rheum. 1990 Aug;33(8):1264–1272. doi: 10.1002/art.1780330829. [DOI] [PubMed] [Google Scholar]
  24. Tervaert J. W., Goldschmeding R., Elema J. D., van der Giessen M., Huitema M. G., van der Hem G. K., The T. H., von dem Borne A. E., Kallenberg C. G. Autoantibodies against myeloid lysosomal enzymes in crescentic glomerulonephritis. Kidney Int. 1990 Feb;37(2):799–806. doi: 10.1038/ki.1990.48. [DOI] [PubMed] [Google Scholar]
  25. Tervaert J. W., Limburg P. C., Elema J. D., Huitema M. G., Horst G., The T. H., Kallenberg C. G. Detection of autoantibodies against myeloid lysosomal enzymes: a useful adjunct to classification of patients with biopsy-proven necrotizing arteritis. Am J Med. 1991 Jul;91(1):59–66. doi: 10.1016/0002-9343(91)90074-8. [DOI] [PubMed] [Google Scholar]
  26. Tervaert J. W., Mulder A. H., Horst G., Haagsma E. B., Kleibeuker J. H., Kallenberg C. G. Antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis, ulcerative colitis, and autoimmune diseases. Gastroenterology. 1992 Mar;102(3):1090–1091. [PubMed] [Google Scholar]
  27. Tervaert J. W., van der Woude F. J., Fauci A. S., Ambrus J. L., Velosa J., Keane W. F., Meijer S., van der Giessen M., van der Hem G. K., The T. H. Association between active Wegener's granulomatosis and anticytoplasmic antibodies. Arch Intern Med. 1989 Nov;149(11):2461–2465. doi: 10.1001/archinte.149.11.2461. [DOI] [PubMed] [Google Scholar]
  28. Tsokos M., Fauci A. S., Costa J. Idiopathic midline destructive disease (IMDD): a subgroup of patients with the "midline granuloma" syndrome. Am J Clin Pathol. 1982 Feb;77(2):162–168. doi: 10.1093/ajcp/77.2.162. [DOI] [PubMed] [Google Scholar]
  29. Wiik A. Delineation of a standard procedure for indirect immunofluorescence detection of ANCA. APMIS Suppl. 1989;6:12–13. [PubMed] [Google Scholar]
  30. ter Borg E. J., Horst G., Hummel E. J., Limburg P. C., Kallenberg C. G. Measurement of increases in anti-double-stranded DNA antibody levels as a predictor of disease exacerbation in systemic lupus erythematosus. A long-term, prospective study. Arthritis Rheum. 1990 May;33(5):634–643. doi: 10.1002/art.1780330505. [DOI] [PubMed] [Google Scholar]

Articles from Annals of the Rheumatic Diseases are provided here courtesy of BMJ Publishing Group

RESOURCES