Test Catalog

Test Id : PR3

Proteinase 3 Antibodies, IgG, Serum

Useful For
Suggests clinical disorders or settings where the test may be helpful

Evaluating patients suspected of having Wegener granulomatosis (WG)

 

Distinguishing between WG and other forms of vasculitis, in conjunction with:

-MPO / Myeloperoxidase Antibodies, IgG, Serum

-ANCA / Cytoplasmic Neutrophil Antibodies, Serum (may be obtained as VASC / Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum)

 

May be useful to follow treatment response or to monitor disease activity in patients with myeloperoxidase antibodies

Method Name
A short description of the method used to perform the test

Multiplex Flow Immunoassay

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

Proteinase 3 Ab (PR3), S

Aliases
Lists additional common names for a test, as an aid in searching

ACPA (Antineutrophil Cytoplasmic Antibodies)

ANCA (Antineutrophil Cytoplasmic Antibodies)

Anticytoplasmic Autoantibodies

Antineutrophil Cytoplasmic Antibodies (ACPA)

Autoantibodies to Proteinase 3

c-ANCA (Antineutrophil Cytoplasmic Antibodies)

cANCA (Antineutrophil Cytoplasmic Antibodies)

Cytoplasmic Neutrophil Antibodies

Neutrophil Cytoplasmic Antibodies

pANCA (Perinuclear Antineutrophil Cytoplasmic Antibodies)

Perinuclear Antineutrophil Cytoplasmic Antibodies (pANCA)

PR3 (Proteinase 3 Ab), S

PR3 (Proteinase 3) Antineutrophil Cytoplasmic Antibodies

PR3 ANCA

Proteinase 3 (PR3) Antineutrophil Cytoplasmic Antibodies

Wegener's Granulomatosis

Specimen Type
Describes the specimen type validated for testing

Serum

Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

Container/Tube:

Preferred: Serum gel

Acceptable: Red top

Specimen Volume: 0.5 mL

Forms

If not ordering electronically, complete, print, and send a Renal Diagnostics Test Request (T830) with the specimen.

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory

0.35 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis Reject
Gross lipemia Reject
Gross icterus OK

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

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

Useful For
Suggests clinical disorders or settings where the test may be helpful

Evaluating patients suspected of having Wegener granulomatosis (WG)

 

Distinguishing between WG and other forms of vasculitis, in conjunction with:

-MPO / Myeloperoxidase Antibodies, IgG, Serum

-ANCA / Cytoplasmic Neutrophil Antibodies, Serum (may be obtained as VASC / Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum)

 

May be useful to follow treatment response or to monitor disease activity in patients with myeloperoxidase antibodies

Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Proteinase 3 (PR3) antigen is a 29-kD serine protease that exists as a protein triplet in human neutrophils.

 

Wegener granulomatosis (WG) is an autoimmune vasculitis that affects the kidneys and lungs, as well as other organs. Patients with WG develop autoantibodies to the PR3 antigen of myeloid lysosomes (PR3 antineutrophil cytoplasmic antibodies [PR3 ANCA]).(1)

 

Since it is often impossible to distinguish between WG and other forms of vasculitis on the basis of clinical signs and symptoms, tests for PR3 ANCA should be employed with other serologic tests in the initial diagnostic evaluation of patients with clinical features of vasculitis (eg, VASC / Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum).

Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

<0.4 U (negative)

0.4-0.9 U (equivocal)

> or =1.0 U (positive)

Reference values apply to all ages.

Interpretation
Provides information to assist in interpretation of the test results

Proteinase 3 antineutrophil cytoplasmic antibodies (PR3 ANCA) are detectable in nearly all patients with severe active Wegener granulomatosis (WG).(2) The presence of PR3 ANCA is a specific diagnostic indicator of WG; less than 2% of positive results occur in patients who do not have the disease.(3,4)

 

A negative result for PR3 ANCA diminishes the likelihood that a patient has active WG; but, approximately 20% of patients with limited WG may test negative for PR3 ANCA.(3)

 

The levels of PR3 ANCA often decline following successful treatment of patients with WG. Nevertheless, follow-up testing for PR3 ANCA to evaluate clinical status in treated patients should be used with caution as the levels of antibodies may correlate poorly with clinical status in some patients.

Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

While the presence of proteinase 3 antineutrophil cytoplasmic antibodies (PR3 ANCA) is highly specific for Wegener granulomatosis (WG), it is recommended that positive test results obtained by immunoassay be confirmed by another testing method.(4) This is best accomplished by testing for cytoplasmic ANCA (cANCA) and perinuclear ANCA (pANCA) by indirect immunofluorescence microscopy (ANCA / Cytoplasmic Neutrophil Antibodies, Serum). Alternately, VASC / Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum includes tests for PR3 ANCA, myeloperoxidase antibodies, and, if indicated, cANCA and pANCA. This panel is recommended for the initial diagnostic evaluation of patients clinically suspected of having systemic vasculitis. The simultaneous presence of PR3 ANCA and cANCA has a specificity greater than 99% for WG.(3)

Clinical Reference
Recommendations for in-depth reading of a clinical nature

1. van der Woude FJ, Rasmussen N, Lobatto S, et al: Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener granulomatosis. Lancet 1985;1:425-429

2. Finkleman JD, Lee AS, Hummel AM, et al: ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis. Am J Med 2007;120:643

3. Russel KA, Wiegert E, Schroeder DR, et al: Detection of anti-neutrophil cytoplasmic antibodies under actual clinical testing conditions. Clin Immunol 2002;103:196-203

4. 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;111:507-513

Method Description
Describes how the test is performed and provides a method-specific reference

Proteinase 3 (PR3) antigen is covalently coupled to polystyrene microspheres that are impregnated with fluorescent dyes to create a unique fluorescent signature. PR3 antibodies, if present in diluted serum, bind to the PR3 antigen on the microspheres. The microspheres are washed to remove extraneous serum proteins. Phycoerythrin (PE)-conjugated antihuman IgG antibody is then added to detect IgG anti-PR3 bound to the microspheres. The microspheres are washed to remove unbound conjugate, and bound conjugate is detected by laser photometry. A primary laser reveals the fluorescent signature of each microsphere to distinguish it from microspheres that are labeled with other antigens. A secondary laser reveals the level of PE fluorescence associated with each microsphere. Results are calculated by comparing the median fluorescence response for PR3 microspheres to a 4-point calibration curve.(Package insert: Bio-Plex 2200 Vasculitis. Bio-Rad Laboratories, Hercules, CA 4/2012)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

Monday through Saturday

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

Same day/1 to 3 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

14 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.

  • Authorized users can sign in to Test Prices for detailed fee information.
  • Clients without access to Test Prices can contact Customer Service 24 hours a day, seven days a week.
  • Prospective clients should contact their Regional Manager. For assistance, contact Customer Service.

Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

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.

CPT Code Information
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

83516

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

Test Id Test Order Name Order LOINC Value
PR3 Proteinase 3 Ab (PR3), S 74106-6
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.
PR3 Proteinase 3 Ab (PR3), S 74106-6

Test Setup Resources

Setup Files
Test setup information contains test file definition details to support order and result interfacing between Mayo Clinic Laboratories and your Laboratory Information System.

Excel | Create a PDF

Sample Reports
Normal and Abnormal sample reports are provided as references for report appearance.

Normal Reports | Abnormal Reports

SI Sample Reports
International System (SI) of Unit reports are provided for a limited number of tests. These reports are intended for international account use and are only available through MayoLINK accounts that have been defined to receive them.

SI Normal Reports | SI Abnormal Reports