Antiproteinase 3 (PR3) Antibodies

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Category:

Turnaround Time: 1 - 3 days
CPT Code:

83520

Test Type: 0.6 mL Serum
Stability Time:

Temperature

Period

Room temperature

14 days

Refrigerated

14 days

Frozen

14 days

Freeze/thaw cycles

Stable x1

Reference Range:

0.0-3.5 units/mL

Overview:

For diagnosis and monitoring inflammatory activity in primary systemic small vessel vasculitides. Antineutrophil antibodies are best demonstrated in these diseases by using a combination of IFA and EIAs that detect ANCA specific for PR3-ANCA or MPO-ANCA. Presence of anti-MPO antibodies are highly specific for idiopathic and vasculitis associated crescentic glomerulonephritis, classic polyarteritis nodosa, Churg-Strauss syndrome, and polyangiitis overlap syndrome without renal involvement.

Presence of anti-PR3 antibodies are highly specific for Wegener granulomatous (WG) disease, for which the sensitivity is reported to be 98%. Some patients with WG have pANCA with MPO specificity. About 60% of patients with microscopic polyangiitis or pauci-immune segmental necrotizing glomerulonephritis have pANCA with MPO specificity but 30% have cANCA with PR3 specificity. Levels of anti-PR3 or anti-MPO are elevated during active phases of disease and lower during remission and can be monitored for management of disease.

Results of this assay are not diagnostic proof of the presence or absence of disease and should be used in conjunction with clinical findings and other serological tests.

De'Oliviera J, Gaskin G, Dash A, Rees AJ, Pusey CD. Relationship between disease activity and anti-neutrophil cytoplasmic antibody concentration in long-term management of systemic vasculitis. Am J Kidney Dis. 1995 Mar; 25(3):380-389. PubMed 7872315

Gross W, Csernok E. Immunodiagnostic and pathophysiologic aspects of antineutrophil cytoplasmic antibodies in vasculitis. Curr Opin Rheumatol. 1995 Jan; 7(1):11-19 (review). PubMed 7718417

Jennette JC, Falk RJ. Antineutrophil cytoplasmic antibodies: Discovery, specificity, disease associations and pathogenic potential. Adv Pathol Lab Med. 1995; 8:363-378.

Savige J, Gillis D, Benson E, et al. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol. 1999 Apr; 111(4):507-513. PubMed 10191771

Collection Details:

Collection Instructions:

Red-top tube or gel-barrier tube.

Room Temperature.