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Anti-Cytoplasmic Antibodies in Wegener’s Granulomatosis are Directed Against Proteinase 3

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New Aspects of Human Polymorphonuclear Leukocytes

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 297))

Abstract

Wegener’s granulomatosis (WG), a systemic necrotizing granulomatous vasculitis, used to be considered a relatively rare disease, but in the past few years it has been diagnosed much more frequently. This is at least partially due to the study by Van der Woude et al. (1985), who showed that autoantibodies directed against a cytoplasmic antigen of human neutrophil granulocytes and monocytes (anti-cytoplasmic antibodies = ACPA, synonym: ANCA; C-ANCA) are a disease-specific marker of WG. The im-munodiagnostic value of ACPA has been confirmed by many investigators (Gross et al., 1986; Savage et al., 1987; Lüdemann and Gross, 1987; Parlevliet et al., 1988). Moreover, it has been shown that changes in ACPA titer parallel changes in disease activity (Van der Woude et al., 1985; Parlevliet et al., 1988; Lüdemann et al., 1988a; Specks et al., 1989; Nölle et al., 1989).

This work was supported by the “Bundesministerium für Forschung und Technologie” (grant Ol VM 8622) and the “Verein zur Förderung der Erforschung und Bekämpfung rheumatischer Erkrankungen Bad Bramstedt e.V.”

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Lüdemann, J., Utecht, B., Gross, W.L. (1991). Anti-Cytoplasmic Antibodies in Wegener’s Granulomatosis are Directed Against Proteinase 3. In: Hörl, W.H., Schollmeyer, P.J. (eds) New Aspects of Human Polymorphonuclear Leukocytes. Advances in Experimental Medicine and Biology, vol 297. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-3629-5_12

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  • DOI: https://doi.org/10.1007/978-1-4899-3629-5_12

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