Rheumatology Book

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Antineutrophil Cytoplasmic AntibodyAka: Antineutrophil Cytoplasmic Antibodies, ANCA, Proteinase 3-ANCA, PR3-ANCA, Myeloperoxidase-ANCA, MPO-ANCA

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  1. See Also
    1. Antinuclear Antibody
    2. Small Vessel Vasculitis
  2. Indications
    1. Small Vessel Vasculitis
    2. Wegener's Granulomatosis suspected (cANCA)
  3. Mechanism
    1. Antibodies bind to cytoplasmic region of Neutrophils
  4. Testing
    1. Cytospin preparations of Neutrophils are permeabilized
    2. Binding of serum antibodies assessed as with ANA
  5. Results
    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)
  6. 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-Strauss Vasculitis (Moderately associated)
      4. Wegener's Granulomatosis (Weakly associated)
      5. Henoch-Schonlein Purpura (Possibly associated)
      6. Temporal Arteritis (Possibly associated)
  7. 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
    4. Lane (2002) Am Fam Physician 65(6):1073

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