Abstract：
:Antineutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of some idiopathic systemic vasculitides, such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and, to a lesser extent, Churg-Strauss syndrome (CCS), the so-called ANCA-associated vasculitides (AAV). ANCA were first detected by immunofluorescence (IIFT), subsequently the target antigens myeloperoxidase (MPO) and proteinase 3 (PR3) were identified, allowing the development of the quantitative, antigen-specific assays. According to the guidelines, combining IIFT and PR3-ANCA/MPO-ANCA assures the optimal diagnostic specificity. Antigen specificity does not effectively differentiate among the different AAV, however C-ANCA/PR3-ANCA are mainly found in GPA, while P-ANCA/MPO-ANCA are more prevalent in MPA and CSS. Despite their diagnostic value, the performance of the widespread immunometric assays for ANCA testing is disappointing, particularly for the low sensitivity. In recent years, more "sensitive" assays have been developed, using the microplate as well as fully the automated technologies, with promising preliminary results. ANCA, may be detected in a number of pathological conditions other than small vessel vasculitis. However, in most of these non-vasculitic patients ANCA do not recognize MPO or PR3 as target antigens, but other granulocyte components, often multiple or unknown specificities. A positive ANCA result by itself is not diagnostic for AAV, clinical evidence and possibly histological confirmation are always required. On the other hand, a negative test result cannot completely rule out a diagnosis of AAV, as AAV without detectable ANCA exist. The appropriate use of ANCA testing strongly improves the diagnostic accuracy and clinical usefulness of the results.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Radice A,Bianchi L,Sinico RAdoi
10.1016/j.autrev.2012.08.008subject
Has Abstractpub_date
2013-02-01 00:00:00pages
487-95issue
4eissn
1568-9972issn
1873-0183pii
S1568-9972(12)00172-3journal_volume
12pub_type
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pub_type： 杂志文章,评审
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pub_type： 杂志文章,评审
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pub_type： 杂志文章
doi：10.1016/j.autrev.2008.05.002
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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