Key Points
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Screening for antineutrophil cytoplasmic antibodies (ANCAs) specific for proteinase 3 (PR3) and myeloperoxidase (MPO) remains a basic tool in the serological evaluation of systemic vasculitic disorders
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Automated analysis of ANCA immunofluorescence demonstrates good agreement with conventional techniques when assessing patients with suspected vasculitis and, in the future, might improve efficiency and standardization in clinical laboratories
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Antigen-specific ANCA assays that use bridging molecules exhibit superior performance compared with conventional assays and can be used for PR3-ANCA and MPO-ANCA detection
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The new methods for ANCA detection and evaluation in ANCA-associated vasculitis should be urgently evaluated in multicentre studies, in anticipation of updating of standardization processes and a revision of existing strategies
Abstract
Detection of antineutrophil cytoplasmic antibodies (ANCAs) is a well-established diagnostic test used to evaluate suspected necrotizing vasculitis of small blood vessels. Conditions associated with these antibodies, collectively referred to as ANCA-associated vasculitides, include granulomatosis with polyangiitis (formerly known as Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly known as Churg–Strauss syndrome). The diagnostic utility of ANCA testing depends on the type of assay performed and on the clinical setting. Most laboratories worldwide use standard indirect immunofluorescence tests (IFT) to screen for ANCA and then confirm positive IFT results with antigen-specific tests for proteinase 3 (PR3) and myeloperoxidase (MPO). Developments such as automated image analysis of immunofluorescence patterns, so-called third-generation PR3-ANCA and MPO-ANCA ELISA, and multiplex technology have improved the detection of ANCAs. However, challenges in routine clinical practice remain, including methodological aspects of IFT performance, the diverse antigen-specific assays available, the diagnostic value of testing in clinical settings and the prognostic value of serial ANCA monitoring in the prediction of disease relapse. This Review summarizes the available data on ANCA testing, discusses the usefulness of the various ANCA assays and advises on the clinical indications for the use of ANCA testing.
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E.C. researched data for and wrote the article, F.M. made a substantial contribution to discussion of content, and both authors reviewed/edited the manuscript before submission.
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Csernok, E., Moosig, F. Current and emerging techniques for ANCA detection in vasculitis. Nat Rev Rheumatol 10, 494–501 (2014). https://doi.org/10.1038/nrrheum.2014.78
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DOI: https://doi.org/10.1038/nrrheum.2014.78
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