Anticytoplasmic Autoantibodies in the Diagnosis and Follow-Up of Wegener's Granulomatosis

ULRICH SPECKS, CHRISTINE L. WHEATLEY, THOMAS J. McDONALD, MICHAEL S. ROHRBACH, RICHARD A. DeREMEE

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221 Scopus citations

Abstract

Sixty-five patients with biopsy-proven Wegener's granulomatosis (WG), 54 with systemic vasculitis, 22 with relapsing polychondritis, 20 with sarcoidosis, 20 with malignant pulmonary lesions, and 15 with other conditions underwent determination of anticytoplasmic autoantibodies (ACPA) by the indirect immunofluorescence technique on neutrophil cytospin preparations to assess the specificity of ACPA for WG, their sensitivity in relationship to the extent and activity of the disease, and their value for follow-up of WG. Of these 65 patients with WG, 38 were ACPA positive. Two patients in the vasculitis group, best categorized as having microscopic polyarteritis, were ACPA positive. We obtained 125 serum samples from the 65 patients with WG and assigned them to one of two categories (limited or generalized), based on the extent of disease. Each of these categories was then subdivided into “active” or “in remission.” Median ACPA titers were significantly different between active disease and remission in each category, as well as between active limited and active generalized disease. All patients whose disease changed from active to in remission had reductions in ACPA titer levels; those who experienced flares had titer increases. Patients with intercurrent illnesses or complications of treatment, mimicking WG flares, did not have titer increases. We conclude that ACPA determined by the indirect immunofluorescence technique is highly specific for WG. The sensitivity is dependent on the extent and activity of WG, and serial titer determinations are valuable in monitoring disease activity.

Original languageEnglish (US)
Pages (from-to)28-36
Number of pages9
JournalMayo Clinic proceedings
Volume64
Issue number1
DOIs
StatePublished - Jan 1 1989

ASJC Scopus subject areas

  • Medicine(all)

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    SPECKS, ULRICH., WHEATLEY, CHRISTINE. L., McDONALD, THOMAS. J., ROHRBACH, MICHAEL. S., & DeREMEE, RICHARD. A. (1989). Anticytoplasmic Autoantibodies in the Diagnosis and Follow-Up of Wegener's Granulomatosis. Mayo Clinic proceedings, 64(1), 28-36. https://doi.org/10.1016/S0025-6196(12)65300-2