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Rapid and accurate testing is essential to reduce mortality and long-term complications from AAV
Signs and symptoms of AAV vary greatly and are not always indicative of the severity of the disease state.
ANNUAL INCIDENCE
20
per million1
MORTALITY
25%
at five years2
AVISE Vasculitis AAV offers a comprehensive panel of disease-specific biomarkers
c-ANCA (IFA)
Granulomatosis with polyangiitis (GPA)
The c-ANCA pattern produces a granular cytoplasmic pattern with interlobular accentuation on ethanol fixed neutrophils. c-ANCA patterns are associated with necrotizing segmental glomerulonephritis and GPA. 3,4
p-ANCA (IFA)
Microscopic Polyangiitis (MPA)
The p-ANCA pattern produces perinuclear staining with or without nuclear extension. The p-ANCA pattern is commonly detected in patients with MPA and about 40% of patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA).4,5
Atypical p-ANCA (IFA)
Microscopic Polyangiitis (MPA)
The p-ANCA pattern produces perinuclear staining with or without nuclear extension. The p-ANCA pattern is commonly detected in patients with MPA and about 40% of patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA).4,5
Anti-GBM (CIA)
Goodpasture's Syndrome (GPS)
Anti-GBM antibodies are often associated with Goodpasture's syndrome and anti-GBM nephritis.8 A significant proportion of patients with anti-GBM disease are also positive for ANCA.
Anti-MPO (CIA)
Microscopic Polyangiitis (MPA)
Anti-MPO antibodies are primarily associated with MPA and to a lesser extent, found in GPA (6%) and EGPA.3,7 However, anti-MPO antibodies can also be seen in connective tissue disease, IBD, some infections, malignancy, and as a reaction to drugs. Therefore, results should be interpreted with care in light of the clinical findings and workup.6
Anti-PR3 (CIA)
Granulomatosis with polyangiitis (GPA)
Anti-PR3 antibodies are primarily associated with GPA and to a lesser extent, found in MPA (10%) and EPGA.3,7 However, anti-PR3 antibodies can also be seen in connective tissue disease, IBD, some infections, malignancy, and as a reaction to drugs.4 Therefore, results should be interpreted with care in light of the clinical findings and workup.6
References
- Draibe JB, Fulladosa X, Cruzado JM, et al. Current and novel biomarkers in anti-neutrophil cytoplasm-associated vasculitis. Clin Kidney J. 2016;9(4):547-551. doi:10.1093/ckj/sfw056
- Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S94-S104.1
- Damoiseaux J, Csernok E, Rasmussen N, et al. Detection of antineutrophil cytoplasmic antibodies (ANCAs): A multicentre European Vasculitis Study Group (EUVAS) evaluation of the value of indirect immunofluorescence (IIF) versus antigen-specific immunoassays. Ann Rheum Dis. 2017;76(4):647-653. doi:10.1136/annrheumdis-2016-209507
- Savige J, Gillis D, Benson E, et al. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol. 1999;111(4):507-13. doi: 10.1093/ajcp/111.4.507
- Bossuyt X, Tervaert JW, Arimura Y, et al. Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheum. 2017;13:683-692. doi: 10.1038/nrrheum.2017.140
- Wallace ZS, Miloslavsky EM. Management of ANCA associated vasculitis. BMJ. 2020;368(March):1-16. doi:10.1136/bmj.m421
- Mahler M, Radice A, Yang W, et al. Clinica Chimica Acta Development and performance evaluation of novel chemiluminescence assays for detection of anti-PR3 and anti-MPO antibodies. Clin Chim Acta. 2012;413(7-8):719-726. doi:10.1016/j.cca.2012.01.004
- Mahler M, Radice A, Sinico RA, et al. Performance evaluation of a novel chemiluminescence assay for detection of anti-GBM antibodies: an international multicenter study. Nephrolog Dial Transplant. 2012;27(1):243-52. doi:10.1093/ndt/gfr203