Bernhard Nolle, MD; Ulrich Specks, MD; Jens Lüdemann, MS; Michael S. Rohrbach, PhD; Richard A. DeRemee, MD; Wolfgang L. Gross, MD
Study Objective: To determine disease specificity and sensitivity of anticytoplasmic autoantibodies (ACPA) for Wegener granulomatosis, as well as their value as a marker of disease activity.
Design: Blind analysis of serum samples, retrospective analysis of clinical data on patients, and prospective follow-up of a subgroup of patients with Wegener granulomatosis.
Patients: The study included 277 patients with Wegener granulomatosis (222 with biopsy-proven disease) and 1657 control patients.
Setting: University hospital and academic medical center.
Laboratory Investigations: Analysis of 2653 serum samples from 1934 patients for ACPA. Antibody detection was by indirect immunofluorescence and a new type of enzyme-linked immunoadsorbent assay (ELISA). Prospective follow-up was on 172 patients with Wegener granulomatosis.
Measurements and Main Results: Specificity of ACPA for Wegener granulomatosis measured by indirect immunofluorescence was 99% (CI, 98.9% to 99.7%) and 98% (CI, 97.4% to 99.2%) by ELISA. Sensitivity of ACPA depended on disease activity and extent: It was 67% (CI, 38% to 89%) by immunofluorescence and 60% (CI, 32% to 84%) by ELISA for patients with active locoregional symptomatology (n = 15); and 32% (CI, 14% to 54%) by immunofluorescence and 40% (CI, 21% to 61%) by ELISA for patients in full remission after initial locoregional symptoms (n = 25). The sensitivity was 96% (CI, 89% to 99%) by immunofluorescence and 93% (CI, 86% to 98%) by ELISA for patients with active generalized disease (n = 92). Serial testing was done; every patient with active generalized disease eventually had at least one positive serum sample. Sensitivity decreased to 41% (CI, 22% to 62%) by both immunofluorescence and ELISA for patients in full remission after active generalized disease (n = 27). Levels of ACPA expressed both as immunofluorescence titers and ELISA values (U/mL) correlated well with disease activity.
Conclusions: Testing for ACPA in serum of patients with Wegener granulomatosis is valuable for differential diagnosis; furthermore, APCA can be used as a marker to follow disease activity. A new type of ELISA yielded the same results as indirect immunofluorescence for the specificity, sensitivity, and correlation with disease activity of ACPA.
Nolle B, Specks U, Lüdemann J, et al. Anticytoplasmic Autoantibodies: Their Immunodiagnostic Value in Wegener Granulomatosis. Ann Intern Med. 1989;111:28–40. doi: https://doi.org/10.7326/0003-4819-111-1-28
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Published: Ann Intern Med. 1989;111(1):28-40.
Autoimmune Kidney Disease, Interstitial Lung Disease, Nephrology, Pulmonary/Critical Care, Rheumatology.
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