Evaluating patients suspected of having autoimmune vasculitis, both Wegener granulomatosis and microscopic polyangiitis
Test Id | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
MPO | Myeloperoxidase Ab, S | Yes | Yes |
PR3 | Proteinase 3 Ab (PR3), S | Yes | Yes |
Test Id | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
ANCA | Cytoplasmic Neutrophilic Ab, S | Yes | No |
If myeloperoxidase antibody or proteinase 3 antibody is > or =0.4 U, then cytoplasmic neutrophilic antibodies will be performed at an additional charge.
Multiplex Flow Immunoassay
ACPA (Antineutrophil Cytoplasmic Antibodies)
ANCA (Antineutrophil Cytoplasmic Antibodies)
ANCA Panel
Antibodies to Myeloperoxidase
Anticytoplasmic Autoantibodies
Antineutrophil Cytoplasmic Antibodies (ACPA)
Autoantibodies to Myeloperoxidase
Autoantibodies to Proteinase 3
Autoimmune Vasculitis
cANCA (Antineutrophil Cytoplasmic Antibodies)
Cytoplasmic Neutrophil Antibodies
Microscopic Polyangiitis (MPA)
MPO (Myeloperoxidase Antibodies)
Myeloperoxidase Antibodies (MPO)
Neutrophil Cytoplasmic Antibodies
pANCA (Perinuclear Antineutrophil Cytoplasmic Antibody)
Pauci-Immune Necrotizing Glomerulonephritis
Perinuclear Antineutrophil Cytoplasmic Antibody (pANCA)
PR3 (Proteinase 3) Antineutrophil Cytoplasmic Antibodies
Proteinase 3 (PR3) Antineutrophil Cytoplasmic Antibodies
Vasculitis Panel
Wegener's Granulomatosis
33185-VASC
If myeloperoxidase antibody or proteinase 3 antibody is > or =0.4 U, then cytoplasmic neutrophilic antibodies will be performed at an additional charge.
Serum
Container/Tube:
Preferred: Serum gel
Acceptable: Red top
Specimen Volume: 1 mL
If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:
-General Request (T239)
-Renal Diagnostics Test Request (T830)
0.5 mL
Gross hemolysis | Reject |
Gross lipemia | Reject |
Gross icterus | OK |
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 21 days | |
Frozen | 21 days |
Evaluating patients suspected of having autoimmune vasculitis, both Wegener granulomatosis and microscopic polyangiitis
If myeloperoxidase antibody or proteinase 3 antibody is > or =0.4 U, then cytoplasmic neutrophilic antibodies will be performed at an additional charge.
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.
MYELOPEROXIDASE ANTIBODIES, IgG
<0.4 U (negative)
0.4-0.9 U (equivocal)
> or =1.0 U (positive)
Reference values apply to all ages.
PROTEINASE 3 ANTIBODIES, IgG
<0.4 U (negative)
0.4-0.9 U (equivocal)
> or =1.0 U (positive)
Reference values apply to all ages.
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)
Sequential measurements of titers of antineutrophil cytoplasmic antibodies (cANCA) are useful to monitor the response to treatment in patients with Wegener granulomatosis (WG). While titers often decrease following successful treatment, the results cannot be relied upon in all cases to determine the response to therapy. In individual patients, the titers of cANCA may not correlate well with response to treatment. The results of proteinase 3 (PR3) ANCA (an autoantibody with a cANCA pattern) by EIA have not been shown to be useful for monitoring disease activity. Accordingly, for monitoring disease activity we advise physicians to order ANCA / Cytoplasmic Neutrophil Antibodies, Serum or MPO / Myeloperoxidase Antibodies, IgG, Serum (the ANCA vasculitis test panel is not appropriate for monitoring disease activity).
The ANCA vasculitis test panel cannot be relied upon exclusively to establish the diagnosis of autoimmune vasculitis (WG or microscopic polyangiitis: MPA). Some patients with WG or MPA may not have a measurable titer of antibodies detected by this panel of tests. Some of these patients have antibodies to other neutrophil antigens not included in the panel (eg, neutrophil elastase).
1. Russell KA, Wiegert E, Schroeder DR, et al: Detection of
2. Specks U, Homburger HA, DeRemee RA: Implications of
See Individual Unit Codes
Monday through Saturday
This test has been cleared, approved, or is exempt by the US 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.
83516 x 2
86036 (if appropriate)
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
VASC | ANCA Panel for Vasculitis, S | 90230-4 |
Result Id | Test Result Name |
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
---|---|---|
MPO | Myeloperoxidase Ab, S | 48404-8 |
PR3 | Proteinase 3 Ab (PR3), S | 74106-6 |