Atypical autoantigen targets of perinuclear antineutrophil cytoplasm antibodies (P-ANCA): specificity and clinical associations

J Autoimmun. 1993 Apr;6(2):185-95. doi: 10.1006/jaut.1993.1016.


Atypical antineutrophil cytoplasm antibodies (A-ANCA) are defined here as ANCA detected by IIF and not directed against the predominant ANCA antigens, proteinase 3 (PR3) and myeloperoxidase (MPO). A-ANCA are found in a variety of clinical conditions, namely rheumatoid arthritis, inflammatory bowel diseases, chronic hepatic diseases and several infections including HIV infection. They are directed against a variety of still ill-defined neutrophil antigens and most frequently yield a perinuclear pattern (P-ANCA) of binding by indirect immunofluorescence on ethanol fixed neutrophils. This paper reviews the literature on A-ANCA and our recent data suggesting that, among others, cathepsin G is one of the predominant antigen targets of A-ANCA. From a clinical point of view, the distinction between MPO-ANCA and A-ANCA is not possible by indirect immunofluorescence (IIF). The determination of ANCA antigens by specific ELISA is therefore necessary to differentiate P-ANCA with MPO specificity from those with undefined specificity. This is of importance because the clinical value of MPO-ANCA is clearly established while the presence of A-ANCA is difficult to interpret given their occurrence in a large variety of clinical conditions.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Antibody Specificity
  • Arthritis, Rheumatoid / immunology
  • Autoantibodies / immunology*
  • Autoantigens / immunology*
  • Autoimmune Diseases / immunology*
  • Cathepsin G
  • Cathepsins / immunology
  • Cytoplasm / immunology*
  • Enzyme-Linked Immunosorbent Assay*
  • Fluorescent Antibody Technique
  • Humans
  • Infections / immunology
  • Inflammatory Bowel Diseases / immunology
  • Liver Diseases / immunology
  • Myeloblastin
  • Neutrophils / immunology*
  • Neutrophils / ultrastructure
  • Pancreatic Elastase / immunology
  • Peroxidase / immunology
  • Serine Endopeptidases / immunology


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Autoantigens
  • Peroxidase
  • Cathepsins
  • Serine Endopeptidases
  • CTSG protein, human
  • Cathepsin G
  • Pancreatic Elastase
  • Myeloblastin