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The Role of Antineutrophil Cytoplasmic Antibody (c-ANCA) Testing in the Diagnosis of Wegener Granulomatosis: A Literature Review and Meta-analysis

Jaya K. Rao, MD; Morris Weinberger, PhD; Eugene Z. Oddone, MD; Nancy B. Allen, MD; Pamela Landsman, MPH; and John R. Feussner, MD
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From Durham Veterans Affairs Medical Center and Duke University Medical Center, Durham, North Carolina; and Roudebush Veterans Affairs Medical Center and Indiana University Medical Center, Indianapolis, Indiana. Acknowledgments: The authors thank Gregory Samsa, PhD, and Victor Hasselbad, PhD, for helpful advice; and Theodore Pincus, MD, for his constructive review of earlier drafts of this manuscript. Grant Support: Dr. Rao was supported by the Health Services Research Field Program, Department of Veterans Affairs, and the Department of Veterans Affairs Fellowship Program in Health Services Research. Drs. Oddone and Weinberger are supported by the Department of Veterans Affairs Health Services Research Career Development Program. Requests for Reprints: Dr. Jaya Rao, Health Services Research and Development (11H), Roudebush Veterans Affairs Medical Center, 1481 West 10th Street, Indianapolis, IN 46202. Current Author Addresses: Drs. Rao and Weinberger: Health Services Research & Development (11H), Roudebush Veterans Affairs Medical Center, 1481 West 10th Street, Indianapolis, IN 46202. Drs. Oddone and Feussner: Health Services Research & Development (152), Durham Veterans Affairs Medical Center, 508 Fulton Street, Durham, NC 27705. Dr. Allen: Division of Rheumatology, Box 3440, Duke University Medical Center, Durham, NC 27710. Ms. Landsman: Pfizer, Inc., 219-2-3, 235 East 42nd Street, New York, NY 10017-5755.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1995;123(12):925-932. doi:10.7326/0003-4819-123-12-199512150-00005
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Purpose: To summarize the literature on the clinical utility of antineutrophil cytoplasmic antibody (c-ANCA) as a diagnostic marker for Wegener granulomatosis.

Data Sources: A structured literature search was done using MEDLINE; this search, combined with a manual search, yielded 747 articles for potential inclusion. These articles passed through a 4-stage review. Studies were included if they had a specified reference standard and a systematic method of patient selection and if a 2 × 2 contingency table could be constructed from their results. Fifteen articles met these criteria.

Data Extraction: 3 physicians reviewed all selected articles. Detailed data abstraction was done, and the quality of the study methods was assessed. Items abstracted included the method of patient selection, the study design, the reference standard, the c-ANCA testing technique, disease activity, and the test results. Disagreements among reviewers were resolved by consensus. Contingency tables were used to calculate the operating characteristics for Wegener granulomatosis overall and for active and inactive Wegener granulomatosis.

Results: The sensitivities of c-ANCA testing for overall Wegener granulomatosis ranged from 34% to 92%, and the specificities ranged from 88% to 100%. The pooled sensitivity was 66% (95% CI, 57% to 74%), and the pooled specificity was 98% (CI, 96% to 99.5%). Four articles provided data on disease activity. For active disease, the pooled sensitivity was 91% (CI, 87% to 95%), and the pooled specificity was 99% (CI, 97% to 99.9%). For inactive disease, the pooled sensitivity and specificity were 63% and 99.5%, respectively.

Conclusions: Although c-ANCA test results may serve clinicians as adjunct evidence for the diagnosis of Wegener granulomatosis, these results must be viewed in the context of the patient's clinical picture and disease activity and the prevalence of Wegener granulomatosis in the clinical setting in which the patient is seen.


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Figure 1.
Operating characteristics of c-ANCA testing for confirmed Wegener granulomatosis.Top.Middle.Bottom.

Overall disease. Active disease. Inactive disease.

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