About
ANCA-associated vasculitis are autoimmune diseases characterized by inflammation of small to medium-sized blood vessels, primarily caused by the presence of anti-neutrophil cytoplasmic antibodies (ANCAs). These antibodies target neutrophils, a type of white blood cell, leading to vascular inflammation and subsequent damage to various organs and tissues. The main types of ANCA-associated vasculitis are:
- Granulomatosis with Polyangiitis: previously known as Wegener’s granulomatosis, this condition typically affects the respiratory tract (sinuses, nose, trachea, and lungs) and kidneys. It is characterized by granulomatous inflammation, which are clusters of immune cells.
- Microscopic Polyangiitis: this type primarily affects the kidneys and lungs but can involve other organs, too.
- Eosinophilic Granulomatosis with Polyangiitis: also known as Churg-Strauss syndrome, this condition is marked by asthma, high levels of eosinophils, and granulomatous inflammation, often affecting the lungs, skin, heart, and peripheral nerves.
- Anti-GBM (anti-glomerular basement membrane) glomerulonephritis is a rare autoimmune disease characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM), a critical component of the kidney’s filtering units (glomeruli). This condition can lead to rapidly progressive glomerulonephritis, which is a severe form of kidney inflammation.
- Other autoimmune disorders like Primary Sclerosing Cholangitis, Ulcerative colitis and Felty Syndrome are characterized by the presence of ANCAs antibodies with different target antigens, e.g. Lactoferrin and Cathepsin G, from the most common targets PR3 and MPO.
Diagnosis
The diagnosis of ANCA-associated vasculitis leverages confirmatory test like PR3 (Proteinase 3), MPO (Myeloperoxidase), and GBM (Glomerular Basement Membrane) for a reliable identification of autoantibodies. Various tests such as Lactoferrin, Lysozyme and BPI (bactericidal permeability enhancer protein) are available to aid the diagnosis of other ANCA-related autoimmune diseases that mainly affect the gastrointestinal tract.
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Sebia can support a reliable diagnostic path with Alegria Monotest random access solution, completed by ELISA testing for high throughput. The all-in-one Alegria Monotest is particularly suited for small series and offers unique flexibility for maximum workflow efficiency with the Alegria 2 system.
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- Schirmer, J. H., Wright, M. N., Herrmann, K., & Wieczorek, R. (2020). “Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positive vasculitis: the spectrum of disease.” Journal of Clinical Rheumatology, 26(6), e174-e179.
- Ou CC, Wu YC, Chen JP, Huang WN, Chen YH, Chen YM. Association of atypical anti-neutrophil cytoplasmic antibody with comorbidities and outcome in a hospital-based population. Heliyon. 2024 Jan 6;10(1):e24105. doi: 10.1016/j.heliyon.2024.e24105. PMID: 38234907; PMCID: PMC10792567.
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- Damoiseaux J. ANCA Testing in Clinical Practice: From Implementation to Quality Control and Harmonization. Front Immunol. 2021 Mar 16;12:656796. doi: 10.3389/fimmu.2021.656796. PMID: 33796118; PMCID: PMC8008144.
- Almaani S, Fussner LA, Brodsky S, Meara AS, Jayne D. ANCA-Associated Vasculitis: An Update. J Clin Med. 2021 Apr 1;10(7):1446. doi: 10.3390/jcm10071446. PMID: 33916214; PMCID: PMC8037363.
- Tervaert JW, Damoiseaux J. Fifty years of antineutrophil cytoplasmic antibodies (ANCA) testing: do we need to revise the international consensus statement on testing and reporting on ANCA? APMIS Suppl. 2009 Jun;(127):55-9. doi: 10.1111/j.1600-0463.2009.02478.x. PMID: 19515141.
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