II. Indications

III. Mechanism

  1. Antibodies bind to cytoplasmic region of Neutrophils

IV. Labs

  1. Cytospin preparations of Neutrophils are permeabilized
  2. Binding of serum antibodies assessed as with ANA

V. Labs: Interpretation

  1. C-ANCA (Cytoplasmic: PR3-ANCA)
    1. Classic, Diffuse, fine granular cytoplasmic staining
    2. Antibodies directed to Proteinase-3 antigen (PR3)
  2. P-ANCA (Peri-nuclear: MPO-ANCA)
    1. Perinuclear pattern
    2. Antibodies to Myeloperoxidase (MPO)

VI. Associated Conditions

  1. C-ANCA or PR3-ANCA
    1. Wegener's Granulomatosis (Very Strongly associated)
      1. Positive in 75-98% of patients with Wegener's
      2. Sensitivity drops to 30% during remission
    2. Crescentic Glomerulonephritis (moderately associated)
    3. Polyarteritis Nodosa (Weakly associated)
  2. P-ANCA or MPO-ANCA
    1. Crescentic Glomerulonephritis (Strongly associated)
    2. Polyarteritis Nodosa (Moderately associated)
    3. Churg-StraussVasculitis (Moderately associated)
    4. Wegener's Granulomatosis (Weakly associated)
    5. Henoch-Schonlein Purpura (Possibly associated)
    6. Temporal Arteritis (Possibly associated)

VII. References

  1. Gladman in Klippel (1997) Rheumatic Diseases p. 255-6
  2. Peng in Ruddy (2001) Kelley's Rheumatology, p. 161-72
  3. Callegari (1995) Postgrad Med, 97(4):65-74 [PubMed]
  4. Lane (2002) Am Fam Physician 65(6):1073-80 [PubMed]

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Ontology: Antineutrophil Cytoplasmic Antibodies (C0103647)

Definition (MSH) Autoantibodies directed against cytoplasmic constituents of POLYMORPHONUCLEAR LEUKOCYTES and/or MONOCYTES. They are used as specific markers for GRANULOMATOSIS WITH POLYANGIITIS and other diseases, though their pathophysiological role is not clear. ANCA are routinely detected by indirect immunofluorescence with three different patterns: c-ANCA (cytoplasmic), p-ANCA (perinuclear), and atypical ANCA.
Concepts Immunologic Factor (T129)
MSH D019268
SnomedCT 23318004
LNC LP39202-4, MTHU001866
English ANCA, Anti Neutrophil Cytoplasmic Antibodies, Anti Neutrophil Cytoplasmic Antibody, Anti-Neutrophil Cytoplasmic Antibodies, Anti-Neutrophil Cytoplasmic Antibody, Antibodies, Anti-Neutrophil Cytoplasmic, Antibodies, Antineutrophil Cytoplasmic, Antibody, Anti-Neutrophil Cytoplasmic, Antibody, Antineutrophil Cytoplasmic, Antineutrophil Cytoplasmic Antibodies, Antineutrophil Cytoplasmic Antibody, Cytoplasmic Antibodies, Anti-Neutrophil, Cytoplasmic Antibodies, Antineutrophil, Cytoplasmic Antibody, Anti-Neutrophil, Cytoplasmic Antibody, Antineutrophil, Neutrophil cytoplasmic ab, ANCA - Anti-neutroph cytopl ab, Antineutrophil cytoplasm antib, Antibodies, Antineutrophil Cytoplasmic [Chemical/Ingredient], antineutrophil cytoplasmic antibody (ANCA), anca, Anti-neutrophil cytoplasmic antibodies, Neutrophil cytoplasmic Ab, Neutrophil cytoplasmic Antibody, Anticytoplasmic Neutrophil Antibody, Anti neutrophilic cytoplasm antibody, ANCA - Anti-neutrophil cytoplasmic antibody, Anti-neutrophil cytoplasmic antibody, Neutrophil cytoplasmic antibody, Anti neutrophilic cytoplasm antibody (substance)
Swedish Antineutrofila cytoplasmaantikroppar
Czech protilátky proti cytoplazmě neutrofilů, ANCA, autoprotilátky proti cytoplazmě neutrofilů, protilátky proti cytoplazmě neutrofilních leukocytů, protilátky ANCA
Finnish ANCA-vasta-aineet
Italian ANCA, Anticorpo antineutrofilo anticitoplasma, Anticorpi antineutrofili anticitoplasma
Russian ANTITELA ANTINEITROFIL'NYE TSITOPLAZMATICHESKIE, ANTINEITROFIL'NYE TSITOPLAZMATICHESKIE ANTITELA, C-ANCA, P-ANCA, АНТИНЕЙТРОФИЛЬНЫЕ ЦИТОПЛАЗМАТИЧЕСКИЕ АНТИТЕЛА, АНТИТЕЛА АНТИНЕЙТРОФИЛЬНЫЕ ЦИТОПЛАЗМАТИЧЕСКИЕ
Japanese 抗好中球細胞質抗体, 抗体-好中球細胞質, 抗好中球性細胞質抗体, 抗体-抗好中球細胞質, 抗体-抗ヒト好中球細胞質, 抗好中性細胞質抗体, 好中球細胞質抗体, 抗ヒト好中球細胞質抗体
French Anticorps ANCA, Anticorps anticytoplasme des polynucléaires neutrophiles, Anticorps antineutrophiles cytoplasmiques, Anticorps antineutrophile cytoplasmique, Anticorps anti-neutrophile cytoplasmique, Anticorps anti-neutrophiles cytoplasmiques, Anticorps anti-cytoplasme des polynucléaires neutrophiles, ANCA
Croatian ANTITIJELA, ANTINEUTROFILNA CITOPLAZMATSKA
Polish ANCA, P-ANCA, Przeciwciała cytoplazmatyczne przeciw cytoplazmie neutrofili, C-ANCA, Przeciwciała cytoplazmatyczne antyneutrofilowe, Przeciwciała cytoplazmatyczne przeciwneutrofilowe
Portuguese Anticorpos Anti-Citoplasma de Neutrófilos, ANCA, Anticorpos Anticitoplasma de Neutrófilos
Spanish anticuerpo anti citoplasma neutrofílico (sustancia), anticuerpo anti citoplasma neutrofílico, anticuerpo citoplasmático antineutrófilo, ANCA, Anticuerpos Antineutrófilo Citoplasmáticos, Anticuerpos Citoplasmáticos Antineutrófilo
German ANCA, Antikörper, antineutrophil-cytoplasmatische, Antineutrophil-cytoplasmatische Antikörper