Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegener's granulomatosis

Arthritis Rheum. 1993 Mar;36(3):365-71. doi: 10.1002/art.1780360312.


Objective: To assess the correlation and prognostic value of antineutrophil cytoplasmic antibody (cANCA) titers with disease activity in patients with Wegener's granulomatosis (WG).

Methods: One hundred six patients with WG had serum ANCA determinations; 72 had serial titers obtained routinely at 1-3-month intervals. One hundred twelve subjects (19 of whom were healthy donors) served as controls. All serum samples were tested for cANCA by an indirect immunofluorescence technique. A prospective analysis of disease activity and cANCA values was performed. Disease activity was assessed according to clinical, laboratory, radiographic, and histopathologic findings.

Results: Positivity for cANCA was a sensitive (88%) marker of active WG. However, changes in serial titers temporally correlated with a change in disease status in only 64% of patients. Furthermore, an increase in the cANCA titer preceded clinical exacerbation of disease in only 24% of patients who had been in remission or had low-grade, smoldering disease.

Conclusion: A rise in cANCA titer alone should not be considered adequate evidence of an impending clinical exacerbation, and therefore does not justify initiating or increasing immunosuppressive therapy.

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies / analysis*
  • Biomarkers
  • Cyclophosphamide / therapeutic use
  • False Positive Reactions
  • Fluorescent Antibody Technique
  • Follow-Up Studies
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / immunology*
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Immunoglobulin G / analysis*
  • Predictive Value of Tests
  • Prednisone / therapeutic use
  • Prognosis
  • Prospective Studies
  • Sensitivity and Specificity


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Biomarkers
  • Immunoglobulin G
  • Cyclophosphamide
  • Prednisone