The presence of anti-neutrophil cytoplasmic autoantibodies (ANCA), detected by indirect immunofluorescence, is of high sensitivity and specificity in the diagnosis of Wegener's granulomatosis and related diseases, associated with vasculitis. Titres of immunofluorescence are thought to closely reflect disease activity. In a retrospective series of 266 sera of 23 patients with at least one positive test for ANCA, disease activity was correlated with ANCA, assayed by immunofluorescence and by enzyme-linked immunosorbent assay; with rheumatoid factor and with erythrocyte sedimentation rate. All tests were of limited value in predicting disease activity or relapse. A normal sedimentation rate and, to a lesser extent, a negative result of ANCA-immunofluorescence, were useful in excluding active disease.