Anti–Proteinase 3 (PR3) Antibodies
Test details
Vasculitides comprise a heterogeneous group of disorders characterised by inflammation and necrosis of the walls of arteries and veins of different calibres supplying various body districts. Symptoms include signs of local and systemic inflammation and secondary symptoms arising from reduced tissue perfusion.
The term “ANCA-associated systemic vasculitides” (AAV), recognised by the Chapel Hill Consensus Conference, identifies primary vasculitides with similar clinical and pathological features in which antibodies to neutrophil cytoplasmic constituents are frequently detected.
AAVs are classified into three main forms, based on clinical phenotype and ANCA subtype:
- Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis)
- Microscopic polyangiitis (MPA)
- Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg–Strauss syndrome)
The detection of ANCA-PR3 and/or ANCA-MPO in patients with symptoms suggestive of vasculitis has a very high positive predictive value. The presence of anti-PR3 antibodies is mainly associated with granulomatosis with polyangiitis (GPA).
Sample type
Serum, EDTA plasma, heparin plasma*, citrate plasma
*ELISA and immunoblot tests only
Method
ELISA, immunoblot, ChLIA
Preparation
Fasting for at least 8-12 hours before sampling
Storage conditions
Refer to the Health Service Charter to check storage conditions
Shipping
+2/+8°C
References
Damoiseaux J, Dähnrich C, Rosemann A, Probst C, Komorowski L, Stegeman CA, Egerer K, Hiepe F, van Paassen P, Stöcker W, Schlumberger W, Tervaert JW. A novel enzyme-linked immunosorbent assay using a mixture of human native and recombinant proteinase-3 significantly improves the diagnostic potential for antineutrophil cytoplasmic antibody-associated vasculitis. Ann Rheum Dis. 2009 Feb;68(2):228-33. doi: 10.1136/ard.2007.086579. Epub 2008 Mar 28. PMID: 18375539.
Gross WL, Schmitt WH, Csernok E. ANCA and associated diseases: immunodiagnostic and pathogenetic aspects. Clin Exp Immunol. 1993 Jan;91(1):1-12. doi: 10.1111/j.1365-2249.1993.tb03345.x. PMID: 8419069; PMCID: PMC1554662.
Hagen EC, Ballieux BE, van Es LA, Daha MR, van der Woude FJ. Antineutrophil cytoplasmic autoantibodies: a review of the antigens involved, the assays, and the clinical and possible pathogenetic consequences. Blood. 1993 Apr 15;81(8):1996-2002. PMID: 8471761.
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