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Jan W. Cohen Tervaert, MD; Fokko J. van der Woude, MD; Anthony S. Fauci, MD; Julian L. Ambrus, MD; Jorge Velosa, MD; William F. Keane, MD; Sijtze Meijer, MD; Marijke van der Giessen, PhD; T. Hauw The, MD; Gjalt K. van der Hem, MD; Cees G. M. Kallenberg, MD

Arch Intern Med. 1989;149(11):2461-2465.

Abstract
• 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.

(Arch Intern Med. 1989;149:2461-2465)

Author Affiliations
From the Department of Internal Medicine, University Hospital, Groningen, the Netherlands (Drs Cohen Tervaert, van der Woude, Meijer, van der Giessen, The, van der Hem, and Kallenberg); The Laboratory of Immunoregulation, National Institutes of Health, Bethesda, Md (Drs Fauci and Ambrus); the Department of Internal Medicine, Mayo Clinic, Rochester, Minn (Dr Velosa); and the Nephrology Section, Hennepin County Medical Center, Minneapolis, Minn (Dr Keane).

Footnotes
Accepted for publication May 17, 1989.

Reprint requests to Department of Internal Medicine, University Hospital, Oostersingel 59, 9713 EZ Groningen, the Netherlands (Dr Cohen Tervaert).

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