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Journal of Immunology Research
Volume 2014, Article ID 185416, 12 pages
Review Article

Novel Clinical and Diagnostic Aspects of Antineutrophil Cytoplasmic Antibodies

1Department of Research, INOVA Diagnostics Inc., 9900 Old Grove Road, San Diego, CA 92131-1638, USA
2Microbiology and Virology Institute, San Carlo Borromeo Hospital, Via Pio II 3, 20153 Milan, Italy
3Division of Immunology, University Hospital Marqués de Valdecilla-IFIMAV, Avenida Valdecilla, 39008 Santander, Spain
4Department of Immunology and Internal Medicine, Hospital of Pitie-Salpêtriere, Boulevard de l’Hôpital, 75634 Paris, France
5Department of Medicine, University of Calgary, 3330 Hospital Dr. NW, Calgary, AB, Canada T2N 4N1

Received 4 February 2014; Accepted 6 March 2014; Published 5 June 2014

Academic Editor: Marvin J. Fritzler

Copyright © 2014 Johannes Schulte-Pelkum et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Antineutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of some idiopathic systemic vasculitides. Besides the investigation of ANCA-associated vasculitis (AAV) and constant effort for a standardized nomenclature and classification of the AAV, a main focus of research during the last few years has been to constantly improve the performance of enzyme immunoassays. With the latest so called third generation ELISA, this goal seemed to be fulfilled. The International Consensus Statement on Testing and Reporting of ANCA gave recommendations for standardized strategies for the serological diagnosis of ANCA. New developments now target the system immanent drawbacks of the respective diagnostic methods, be it the need for batching and the long time to result for ELISA, or the high likelihood of error and subjectivity of indirect immunofluorescence (IIF). Random access technology and multiplexing for solid phase assays as well as digital imaging for IIF are tools which may help to expedite and simplify routine diagnostics in the lab and in emergency settings. Recent findings indicate that PR3-ANCA have clinical utility beyond the diagnosis of AAV. PR3-ANCA can also serve as an aid for the differentiation between ulcerative colitis (UC) and Crohn’s disease (CrD) and the stratification of UC patients. This review provides a detailed review of what is known about ANCA and highlights the latest research and state-of-the-art developments in this area.