Proteinase 3 (PR3) Antibodies

Immunology

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Description

C-ANCA ( Cytoplasmic or Classical Staining ANCA ), denotes a granular cytoplasmic staining pattern on ethanol fixed neutrophils, with some interlobular accentuation. C-ANCA 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 C-ANCA specificities have been detected for cationic protein 57 ( CAP57 ) and cathepsin G. C-ANCA positive is suggestive but not diagnostic of Granulomatosis with Polyangiitis ( GPA ), ( formerly known as Wegener's granulomatosis ), microscopic polyangitis ( MPA )( and its renal limited variant ) and Eosinophilic Granulomatosis with Polyangiitis ( EGPA ) ( formerly known as Churg-Strauss syndrome ) [1].

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Indications

Confirmatory test for the presence of anti-PR3 antibodies in ANCA positive samples.

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Sample Type

2mL Serum ( Gel 5mL Yellow tube ). Requests from outside Sheffield: Transport at ambient temperature via Royal Mail or Courier.

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Reference Range

<3 IU/mL = NegativeReference range established by manufacturer and verified in-house.

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Turnaround Time

Within 3 days

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Testing Frequency

Daily

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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]Bossuyt X, et al. Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangitis and microscopic polyangitis. Nature Reviews Rheumatology. 2017. 13; 683-692.

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See Also

ANCA; MPO

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