Clinical Rheumatology

, Volume 37, Issue 4, pp 875–884 | Cite as

Anti-neutrophil cytoplasmic antibodies and their clinical significance

  • Supaporn Suwanchote
  • Muanpetch Rachayon
  • Pongsawat Rodsaward
  • Jongkonnee Wongpiyabovorn
  • Tawatchai Deekajorndech
  • Helen L. Wright
  • Steven W. Edwards
  • Michael W. Beresford
  • Pawinee Rerknimitr
  • Direkrit Chiewchengchol
Review Article
  • 238 Downloads

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.

Keywords

ANCA ANCA-associated vasculitis ANCA staining pattern 

Notes

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.

Compliance with ethical standards

Disclosures

None.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2018

Authors and Affiliations

  • Supaporn Suwanchote
    • 1
  • Muanpetch Rachayon
    • 1
  • Pongsawat Rodsaward
    • 1
  • Jongkonnee Wongpiyabovorn
    • 1
  • Tawatchai Deekajorndech
    • 2
  • Helen L. Wright
    • 3
  • Steven W. Edwards
    • 4
  • Michael W. Beresford
    • 5
    • 6
  • Pawinee Rerknimitr
    • 7
    • 8
  • Direkrit Chiewchengchol
    • 1
    • 8
    • 9
  1. 1.Center of Excellence in Immunology and Immune-mediated diseases, Department of Microbiology, Faculty of MedicineChulalongkorn UniversityBangkokThailand
  2. 2.Division of Pediatric Nephrology, Department of Pediatrics, Faculty of MedicineChulalongkorn UniversityBangkokThailand
  3. 3.Institute of Ageing and Chronic DiseaseUniversity of LiverpoolLiverpoolUK
  4. 4.Institute of Integrative BiologyUniversity of LiverpoolLiverpoolUK
  5. 5.Institute of Translational MedicineUniversity of LiverpoolLiverpoolUK
  6. 6.Department of Pediatric RheumatologyAlder Hey Children’s NHS Foundation TrustLiverpoolUK
  7. 7.Division of Dermatology, Department of Medicine, Faculty of MedicineChulalongkorn UniversityBangkokThailand
  8. 8.Skin and Allergy Research Unit, Department of Medicine, Faculty of MedicineChulalongkorn UniversityBangkokThailand
  9. 9.Center of Excellence in Immunology and Immune-mediated diseases, Faculty of MedicineChulalongkorn UniversityBangkokThailand

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