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Anti-neutrophil cytoplasmic antibodies and their clinical significance

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Abstract

Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay. A positive staining of ANCA can be classified into three main categories based on the staining patterns: cytoplasmic, perinuclear, and atypical. Patients with granulomatosis with polyangiitis (GPA) mostly have a positive cytoplasmic staining pattern (c-ANCA) whilst a perinuclear pattern (p-ANCA) is more common in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) patients. Atypical pattern (a-ANCA) is rarely seen in patients with systemic small-vessel vasculitis but it can be found in other conditions. Here, techniques for ANCA detection, ANCA staining patterns and their clinical significances are reviewed.

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Acknowledgements

We would like to thank Niramol Thammachareonrach, M.Sc., Immunology Unit, Department of Microbiology, Faculty of Medicine, Chulalongkorn University. Special acknowledgement goes to the Center of Excellence in Immunology and Immune-Mediated Diseases, Department of Microbiology, and the Skin and Allergy Research Unit, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

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Authors and Affiliations

  1. Center of Excellence in Immunology and Immune-mediated diseases, Department of Microbiology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

    Supaporn Suwanchote, Muanpetch Rachayon, Pongsawat Rodsaward, Jongkonnee Wongpiyabovorn & Direkrit Chiewchengchol

  2. Division of Pediatric Nephrology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

    Tawatchai Deekajorndech

  3. Institute of Ageing and Chronic Disease, University of Liverpool, Liverpool, UK

    Helen L. Wright

  4. Institute of Integrative Biology, University of Liverpool, Liverpool, UK

    Steven W. Edwards

  5. Institute of Translational Medicine, University of Liverpool, Liverpool, UK

    Michael W. Beresford

  6. Department of Pediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK

    Michael W. Beresford

  7. Division of Dermatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

    Pawinee Rerknimitr

  8. Skin and Allergy Research Unit, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

    Pawinee Rerknimitr & Direkrit Chiewchengchol

  9. Center of Excellence in Immunology and Immune-mediated diseases, Faculty of Medicine, Chulalongkorn University, Rama 4 Road, Pathumwan, Bangkok, 10330, Thailand

    Direkrit Chiewchengchol

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  1. Supaporn Suwanchote
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Correspondence to Direkrit Chiewchengchol.

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Suwanchote, S., Rachayon, M., Rodsaward, P. et al. Anti-neutrophil cytoplasmic antibodies and their clinical significance. Clin Rheumatol 37, 875–884 (2018). https://doi.org/10.1007/s10067-018-4062-x

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  • DOI: https://doi.org/10.1007/s10067-018-4062-x

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