Proteinase 3 (PR3) Antibodies/ ANCA
Code:
PR3
Sample Type:
2mL Serum (Gel 5mL Yellow tube)
Ref Ranges/Units:
IU/mL
<2 IU/mL = Negative Reference range established by
manuafacturer and verified in-house.
Turnaround:
3 Days
Frequency of Analysis: Daily
Special Precautions/Comments:
Method: EliA performed on Phadia Immunocap 250. Calibration: Calibrated against WHO IgG reference preparation 67/086. EQA scheme: UK NEQAS scheme for neutrophil cytoplasmic and GBM antibodies. IQC: Commercial preparation
Interferences: None known
Interpretation: NORMAL result is NEGATIVE. Results are to be reported as follows: NEGATIVE Result: <2 IU/mL. POSITIVE Result: >2 IU/mL.
Additional Information:
N.B. This is part of the ANCA (MPO/PR3)/ Vasculitis screen’
Indication: Screening test for small vessel vasculitis. Seen particularly in Wegener’s granulomatosis (WG), microscopic polyangitis (MPA) and its renal limited variant (pauci-immune crescentic glomerulonephritis) and Churg-Strauss syndrome.
Background Information: cANCA (Cytoplasmic or Classical Staining ANCA), denotes a granular cytoplasmic staining pattern on
ethanol fixed neutrophils, with some interlobular accentuation. cANCA are principally directed against a 29,000 Dalton serine protease, proteinase 3 (PR3) present in the azurophil granules in the cytoplasm of human neutrophils. Other cANCA specificities have been detected for cationic protein 57 (CAP57) and cathepsin G. cANCA positive is suggestive but not diagnostic of WG, MPA (and its renal limited variant) and Churg-Strauss syndrome [1]
References: Lapraik C, et al. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology. 2007. 46 (10):1615-1616. Bosch X, Guilabert A and Font J. Antineutrophil cytoplasmic antibodies. Lancet. 2006. 368(9533):404-418. Seo P and Stone J. The Antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004. 117:39-50. Reumaux D, Duthilleul P, Roos D. Pathogenesis of diseases associated with antineutrophil cytoplasm autoantibodies. Hum Immunol. 2004. 65(1):1-12. Savige J, et al. Addendum to the International Consensus Statement on testing and reporting of
antineutrophil cytoplasmic antibodies. Quality control guidelines, comments, and recommendations for testing in other autoimmune diseases. Am. J. Clin.Pathol. 2003. 120(3):312-318. Review. [Ref 1]
See Also: ANCA; MPO
Telephone Gateshead Lab: 0191.4456499 Option 4, Option 1