Clinical significance of antiproteinase 3 antibody positivity in cANCA-positive patients

Clin Rheumatol. 1999;18(4):279-82. doi: 10.1007/s100670050102.

Abstract

We addressed the clinical significance of antiproteinase 3 (anti-PR3) antibody (Ab) positivity by reviewing the files of 79 patients whose serum contained antineutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (cANCA) and had been tested for anti-PR3 reactivity. Vasculitis was present in most (22/35) cANCA+ PR3+ patients but in only a few (5/44) cANCA+ PR3- patients, thereby suggesting that anti-PR3 Ab positivity in cANCA+ patients is more indicative of vasculitis than cANCA positivity alone. Noteworthy, one-third of cANCA+ PR3+ patients -- those with anti-PR3 Ab titres lower than 100 U/ml -- did not suffer from vasculitis. Anti-PR3 reactivity in vasculitis patients was only weakly associated with Wegener's granulomatosis (WG), as nine out of 22 cANCA+ PR3+ vasculitis patients (41%) did not fulfil the ACR classification criteria for WG. There was no correlation between anti-PR3 Ab titres and disease activity at diagnosis. However, titres measured when patients were in remission were much lower than initial values. Taken together, our results indicate that anti-PR3 Ab positivity should be interpreted in its clinical context.

Publication types

  • Comparative Study

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Autoantibodies / analysis*
  • Autoantigens / immunology*
  • Biomarkers / blood
  • Diagnosis, Differential
  • Enzyme-Linked Immunosorbent Assay
  • Granulomatosis with Polyangiitis / blood
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / immunology*
  • Humans
  • Myeloblastin
  • Retrospective Studies
  • Serine Endopeptidases / immunology*
  • Severity of Illness Index

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Autoantigens
  • Biomarkers
  • Serine Endopeptidases
  • Myeloblastin