Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis

Hilhorst, Marc; Paassen, Pieter van; Tervaert, Jan Willem Cohen

doi:10.1681/asn.2014090903

Cited by 168 publications

(144 citation statements)

References 172 publications

(149 reference statements)

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“…. The fact that different approaches were needed to demonstrate the pathogenic potential of MPO-ANCAs and PR3-ANCAs in these studies increased the awareness that instead of distinguishing between patients with GPA, MPA and EGPA, differentiating between patients with MPOANCAs or PR3-ANCAs might be more clinically relevant [34][35][36][37] . This notion was underscored in 2012 by the finding that these autoantibodies can be used to differentiate between genetically distinct subsets of patients with AAV 38 .…”

Section: Historical Perspectivementioning

confidence: 99%

Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis

et al. 2017

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| Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications. As dependable immunoassays for PR3-ANCAs and MPO-ANCAs have become broadly available, there is increasing international agreement that high-quality immunoassays are the preferred screening method for the diagnosis of ANCA-associated vasculitis. The present Consensus Statement proposes that high-quality immunoassays can be used as the primary screening method for patients suspected of having the ANCA-associated vaculitides GPA and MPA without the categorical need for IIF, and presents and discusses evidence to support this recommendation.NATURE REVIEWS | RHEUMATOLOGY VOLUME 13 | NOVEMBER 2017 | 683 CONSENSUS STATEMENT © 2 0 1 7 M a c m i l l a n P u b l i s h e r s L i m i t e d , p a r t o f S p r i n g e r N a t u r e . A l l r i g h t s r e s e r v e d .Given the improvements in antigen-specific immuno assays, the international consensus on the testing of ANCAs in small-vessel vasculitis seems in need of updating [7][8][9][10] . In this manuscript, a revised 2017 international consensus is proposed by a group of international experts (from North and Central America, Australia, Europe and Asia) in the ANCA field. This Consensus Statement highlights the value of ANCA testing as a tool for diagnosis (but not follow-up) of GPA and MPA and gives a historical perspective of ANCAs in small-vessel vasculitis. This Consensus Statement does not, however, present evidence-based guidelines or a meta-analysis. MethodsThis Consensus Statement was prepared by a group of experts from four European laboratories (X.B., J.D., N.R., J.W.C.T. and E.C.) in person and by correspondence. The draft was circulated to each contributor and modified, and the resulting document was distributed by e-mail to 16 experts from four continents, selected based on their expertise and knowledge in clinical and/or laboratory aspects of AAV. This revised document resulted in a second round of discussions and revisions. The final document was returned to all contributors for ratification.The Consensus Statement is based on the results of a multicentre European Vasculitis Study Group (EUVAS) evaluation of the value of IIF versus antigen-specific immunoassays for ANCA detection 6,11,12 . This study, which showed a large variability between different IIF methods and a good diagnostic performance of PR3-ANCA and MPO-ANCA immunoassays 6 , indicated that the 1999 international consensus on ANCA testing for AAV needed revision. When the consensus was put toget...

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Section: Historical Perspectivementioning

confidence: 99%

Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis

et al. 2017

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“…The conventional classification based on clinical phenotype has been challenged as there is now good epidemiological, genetic and clinical evidence to support a division based on ANCA subtype. 3 GPA, MPA and EGPA have respective annual incidence rates of 2.1-14.4, 2.4-10.1 and 0.5-3.7 per million in Europe, and the prevalence of AAV is estimated at to be 46-184 per million. 4 They are more common in those aged over 60 years and slightly more common in men.…”

Section: Classification and Epidemiologymentioning

confidence: 99%

ANCA-associated vasculitis

2017

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The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The ANCA-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA - formerly known as Wegener’s granulomatosis); microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA - previously known as Churg-Strauss Syndrome). This review examines recent developments in the pathogenesis and treatment of AAV

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“…Importantly, no difference in the relapse risk between GPA and MPA was observed if patients were stratified according to their ANCA specificity. These findings, in addition to distinct genetic differences between PR3-ANCA-and MPO-ANCA-positive patients but less between patients with GPA and MPA [47], lead to the suggestion that patients should be classified based on ANCA specificity and not the disease entity [2,48,49]. Differences between PR3-ANCA and MPO-AAV have been extensively reviewed elsewhere [49].…”

Section: Patient Classifications -Based On the Chance To Relapsementioning

confidence: 99%

“…These findings, in addition to distinct genetic differences between PR3-ANCA-and MPO-ANCA-positive patients but less between patients with GPA and MPA [47], lead to the suggestion that patients should be classified based on ANCA specificity and not the disease entity [2,48,49]. Differences between PR3-ANCA and MPO-AAV have been extensively reviewed elsewhere [49]. The risk to develop a relapse is not only associated with clinical or serological phenotypes, differences in the genetic background may also play a role [43][44][45].…”

Section: Patient Classifications -Based On the Chance To Relapsementioning

confidence: 99%

“…An ANCA rise is highly associated with a relapse in a subgroup of patients Patients with renal involvement are almost always ANCA positive, whereas a positive ANCA is not always present in patients with localized disease [2,56]. Also, animal models have shown that necrotizing vasculitic lesions are readily induced by ANCA, while granulomas are more difficult to induce [49,103]. These observations led to the hypothesis and the ensuing observation that serial ANCA measurements are highly associated with a relapse in patients with renal involvement (HR 11.09 [94].…”

Section: Serial Anca Measurements -Heterogeneity Between Studiesmentioning

confidence: 99%

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Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Kemna¹,

Paassen²,

Damoiseaux³

et al. 2017

Expert Opinion on Orphan Drugs

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Introduction: Granulomatosis with Polyangiitis (GPA; formerly Wegener's) and Microscopic Polyangiitis (MPA) are inflammatory disease entities affecting small to medium vessels. They are characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA) and are frequently grouped together with Eosinophilic Granulomatosis with Polyangiitis (EGPA; formerly Churg Strauss Syndrome) under the term ANCA-associated Vasculitis (AAV). Due to effective immunosuppressive therapy that was introduced more than 45 years ago, AAV has become a chronic disease in which relapses occur frequently during maintenance treatment or later on after all immunosuppressive therapy is stopped. Since relapses are associated with morbidity and mortality, early detection and prevention of relapses is of great importance. Areas covered: This review focuses on the potential strategies to guide maintenance therapy in patients with GPA or MPA, with an emphasis on patient classifications and the role of serial ANCA measurements. Expert opinion: Risk factors for relapses, e.g., genetic background of the patient, have been studied during the last three decades. Only few of these risk factors directly influence the management of patients. One potentially important factor that may influence therapeutic decisions in AAV patients is serial measurement of ANCA. Recently, it became clear that serial ANCA measurement is an attractive approach in AAV patients with (a history of) capillaritis, e.g., renal involvement or alveolar haemorrhage, but not in patients with more limited disease. Whether ANCA rises can be used to guide therapy andhence-to prevent relapses has been a great debate during the last three decades. At present, we conclude that small studies do support such an approach but that these findings require further validation in larger randomized clinical trials. ARTICLE HISTORY

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Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis

Cited by 168 publications

References 172 publications

Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis

Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis

ANCA-associated vasculitis

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

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