Association between active Wegener's granulomatosis and anticytoplasmic antibodies

Arch Intern Med. 1989 Nov;149(11):2461-5. doi: 10.1001/archinte.149.11.2461.


Autoantibodies reacting with the cytoplasm of granulocytes and monocytes (anticytoplasmic antibodies [ACPAs]) were found in 42 of 45 patients with active Wegener's granulomatosis (WG) (sensitivity, 93%). Specificity was tested in selected groups of patients with closely related diseases. Of 58 patients without a diagnosis of WG, 2 had ACPAs (specificity, 97%). The significance of ACPA titration for assessing or predicting disease activity was evaluated in a 16-month prospective study of 35 patients with WG. Seventeen relapses were observed and all were preceded by a significant rise of the ACPA titer. Anticytoplasmic antibodies are a specific and sensitive marker for active WG; a rising titer is a sensitive marker for the development of a relapse.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Autoantibodies / analysis*
  • Biomarkers / analysis
  • C-Reactive Protein / analysis
  • Cytoplasm / immunology*
  • Female
  • Fluorescent Antibody Technique
  • Granulomatosis with Polyangiitis / blood
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / immunology*
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Male
  • Middle Aged
  • Prospective Studies
  • Sensitivity and Specificity


  • Autoantibodies
  • Biomarkers
  • C-Reactive Protein