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cANCA in body fluid; anti-myeloblastin in body fluid; anti-serine protease 3 in body fluid

Indications: 1) >90% positive predictive value for Wegener's granulomatosis 2) other disorders associated with cANCA include: a) Churg-Strauss syndrome (10%) b) microscopic polyangiitis c) idiopathic cresentic glomeruloneophritis (30%) Clinical significance: 1) directed against myeloblastin (serine proteinase-3) 2) cANCA may play a role in the pathology of Wegener's granulomatosis (see myeloblastin) Specimen: store at -20 C Methods: see anti-neutrophil cytoplasmic antibody (ANCA)

Related

myeloblastin; leukocyte proteinase 3; PR-3; PR3; azurophil granule protein 7; AGP7; Wegener autoantigen; P29; C-ANCA antigen; neutrophil proteinase 4; NP-4 (PRTN3, MBN) neutrophil or polymorphonuclear leukocyte (PMN, poly)

Specific

cANCA in serum; anti-myeloblastin in serum; anti-serine protease 3 in serum proteinase-3 (leukocyte elastase) antibody in body fluid

General

neutrophil cytoplasmic antibody (ANCA) in body fluid

References

  1. Clinical Guide to Laboratory Tests, NW Tietz (ed) 3rd ed, WB Saunders, Philadelphia 1995
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 782, 847
  3. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1913
  4. Kaiser Permanente Northern Calfornia Regional Laboratory
  5. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17. American College of Physicians, Philadelphia 1998, 2012, 2015
  6. Bosch X et al, Antineutrophil cytoplasmic antibodies. Lancet 2006, 368:404 PMID: 16876669