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Test ID: VASC Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum

Reporting Name

ANCA Panel for Vasculitis, S

Useful For

Evaluating patients suspected of having autoimmune vasculitis, both Wegener granulomatosis and microscopic polyangiitis

Clinical Information

Antineutrophil cytoplasmic antibodies (ANCA) occur in patients with autoimmune vasculitis including Wegener granulomatosis (WG), microscopic polyangiitis (MPA), or organ-limited variants thereof such as pauci-immune necrotizing glomerulonephritis.(1) ANCA react with enzymes in the cytoplasmic granules of human neutrophils including proteinase 3 (PR3), myeloperoxidase (MPO), elastase, and cathepsin G.

 

Autoantibodies to PR3 occur in patients with WG (both classical WG and WG with limited end-organ involvement) and produce a characteristic pattern of granular cytoplasmic fluorescence on ethanol-fixed neutrophils called the cANCA pattern. Antibodies to MPO occur predominately in patients with MPA and produce a pattern of perinuclear cytoplasmic fluorescence on ethanol-fixed neutrophils called the pANCA pattern.

 

Autoantibodies to PR3 and MPO can also be detected by EIA methods and are referred to as PR3 ANCA and MPO ANCA, respectively.

Interpretation

Positive results for proteinase 3 (PR3) antineutrophil cytoplasmic antibodies (ANCA) and cANCA or pANCA are consistent with the diagnosis of Wegener granulomatosis (WG), either systemic WG with respiratory and renal involvement or limited WG with more restricted end-organ involvement.

 

Positive results for MPO ANCA and pANCA are consistent with the diagnosis of autoimmune vasculitis including microscopic polyangiitis (MPA) or pauci-immune necrotizing glomerulonephritis.

 

A positive result for PR3 ANCA or MPO ANCA has been shown to detect 89% of patients with active WG or MPA (with or without renal involvement) with fewer than 1% false-positive results in patients with other diseases.(1)

Profile Information

Test ID Reporting Name Available Separately Always Performed
MPO Myeloperoxidase Ab, S Yes Yes
PR3 Proteinase 3 Ab (PR3), S Yes Yes

Reflex Tests

Test ID Reporting Name Available Separately Always Performed
ANCA Cytoplasmic Neutrophilic Ab, S Yes No

Testing Algorithm

If myeloperoxidase antibody or proteinase 3 antibody is ≥0.4 U, then cytoplasmic neutrophilic antibodies will be performed at an additional charge.

Analytic Time

3 days

Day(s) and Time(s) Performed

Monday through Saturday; 4 p.m.

Clinical Reference

1. Russell KA, Wiegert E, Schroeder DR, et al: Detection of anti-neutrophil cytoplasmic antibodies under actual clinical testing conditions. Clin Immunol 2002 May;103(2):196-203

2. Specks U, Homburger HA, DeRemee RA: Implications of cANCA testing for the classifications of Wegner's Granulomatosis: performance of different detection systems. Adv Exp Med Biol 1993;336:65-70

Method Name

Multiplex Flow Immunoassay

Specimen Type

Serum


Advisory Information


For monitoring disease activity, we advise physicians to order ANCA / Cytoplasmic Neutrophil Antibodies, Serum or MPO / Myeloperoxidase Antibodies, IgG, Serum.

Specimen Required


Container/Tube: 

Preferred: Serum gel

Acceptable: Red top

Specimen Volume: 1 mL


Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 21 days
  Frozen  21 days

Reference Values

MYELOPEROXIDASE ANTIBODIES, IgG

<0.4 U (negative)

0.4-0.9 U (equivocal)

≥1.0 U (positive) 

Reference values apply to all ages.

 

PROTEINASE 3 ANTIBODIES, IgG

<0.4 U (negative)

0.4-0.9 U (equivocal)

≥1.0 U (positive) 

Reference values apply to all ages.

Test Classification

This test has been cleared, approved or is exempt by the U.S. Food and Drug Administration and is used per manufacturer's instructions. Performance characteristics were verified by Mayo Clinic in a manner consistent with CLIA requirements.

CPT Code Information

83516 x 2

86255-Cytoplasmic neutrophil antibodies screen (if appropriate)

86256-Cytoplasmic neutrophil antibodies titer (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
VASC ANCA Panel for Vasculitis, S 90230-4

 

Result ID Test Result Name Result LOINC Value
MPO Myeloperoxidase Ab, S 48404-8
PR3 Proteinase 3 Ab (PR3), S 74106-6

Forms

If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:

-General Request (T239)

-Renal Diagnostics Test Request (T830)

Mayo Clinic Laboratories | Neurology Catalog Additional Information:

mml-Behavioral, mml-Cerebrovascular, mml-Epilepsy, mml-Headache, mml-Demyelinating-Diseases, mml-Neuroimmunology, mml-Neuromuscular, mml-Autonomic, mml-Pediatric, mml-Spinal-Cord, mml-Neuro-ophthalmology