AUTOIMMUNE VASCULITIS  TESTS
(Last update 15 May 2011)

Activity of Wegener's granulomatosis has been shown that five disease assessment methods in use yield comparable results for Wegener's granulomatosis and might be extrapolatable to include some other AVs.

This web page has eight sections.  To go to any desired section, click on the section title below.

1 - MEDICAL TEST PHASES    (See Phase 2 for a link to Baseline Testing)
2 - DIAGNOSTIC PROCEDURES
3 - BLOOD TESTS AND COUNTS
4 -URINE TESTS
5 - IMAGING (RADIOGRAPHIC) TESTS
6 - BIOPSY
7 - OTHER TESTS
8 - SOME LINKS TO WG-RELATED MEDICAL TEST INFORMATION

The most important tests for an autoimmune (AV) patient are:

  Blood tests 
Urine Tests
Imaging Tests
Tuberculosis Test
(if never before tested)
cANCA/pANCA 
Urine Creatinine
X-ray/CT Scan/MRI
as appropriate Note 5
 [Must be done before starting immunosuppressives]
Note 1
anti-PR-3/anti-MPO
Blood & Protein
Echo Cardiogram
 
Note 4

Erythrocyte Sedimentation Rate (ESR or SED)
Blood and protein by dipstick (After remission)
Ultra-sound
as appropriate
 
High Sensitivity C Reactive Protein (CRP)
 24 hr. creatinine clearance
(Best measure of kidney function)
Baseline bone scan
 
 Eosinophilic Cationic Protein (ECP)
Note 3
Urine Microscopic
18F FDG PET/CT
Note 2


Complete blood count (CBC)
with differential

Endoscopic exams
as appropriate

Blood Chemistry Panel

  MRA (large vessel)
  DSA (medium & small
             vessel)

Eotaxin 3   Note 3



  IL-6 and Fibrinogen Note 6


Immunoglobulin E (IgE)




Note 1 - If TB test is positive, the patient must receive appropriate long term antibiotic treatment.

Note 2 -
For large and medium size vessel vasculitides, PET/CT investigations can be performed using a whole-body PET/CT scanner (LSO Hi-REZ Biograph 16, Siemens, Erlangen, Germany) with LSO-detector (PET) and 16-slice multi slice CT (MSCT). The tracer used to detect hypermetabolic areas is [18F]-FDG (fluorodeoxyglucose)PET/CT is a sensitive and specific method for the diagnosis of large vessel vasculitis. PET/CT allows the diagnosis of disease activity and vessel stenosis in the same session.

Note 3 - Possibly useful for evaluating Churg-Strauss or other eosinophilic diseases.

Note 4 - Silent heart damage occcurs in some cases of vasculitis.

Note 5 - For CNSV, MRA (magnetic resonance angiogram) is useful for large vessels.  Medium and small vessels can be imaged using DSA (digital subtraction angiogram).  Contrast enhanced MRA may be useful to avoid biopsy of large vessels.

Note 6 - Recent (2008) work suggests IL-6 and fibrinogen levels may track disease activity levels

These are not the only tests but are key to diagnosing and tracking autoimmune vasculitides (AVs), though perhaps no single test alone is sufficient to rule an AV in or out.  For more on the above tests, see the sections below on blood, urine, and on imaging testing.

I - MEDICAL TEST PHASES

There are four phases to medical testing of a AV patient:

  Phase I - Diagnostic tests.

There is a clinical examination, a medical history, and a period of extensive blood, urine, and imaging tests to allow the physician to conclude the patient indeed has AV, and may uncover other diseases, autoimmune or infectious.

There will usually be one or more imaging tests with may be x-ray, computed tomography scan (CT scan) or magnetic resonance imaging (MRI), or ultra-sound.  An echo-cardiogram is needed to detect silent heart damage.

A bone scan may be done as a baseline to record pretreatment bone density.

Phase 2 - Baseline Testing
To have a record of the patient's condition before starting treatment, physicians may chose to have a
series of baseline tests made. Some baseline information will already be available from the
Diagnostic Tests done during Phase 1.
Click here to view a file on Baseline Testing

  

Phase 3 - Disease tracking during treatment
After begining treatment, there will be frequent blood and urine tests, perhaps weekly.
Once the disease starts to come under control, blood and urine testing will be less frequent, perhaps monthly or bi-monthly.
Imaging tests are likely be at longer intervals than the blood and urine tests.

 ------->  IMPORTANT  <---------
Persons taking long-term TNF[alpha]-suppressing drugs such as Enbrel, Remicade, and Humira or Rituxan, should request an annual purified protein derivative (PPD) testing to detect latent TB infections if the patient has been exposed to persons with TB, or traveled.
  Phase 4 - Follow-up after the patient is in remission.
Once in remission, blood and urine tests may be several months apart unless symptoms indicate a possible relapse.

As long as a person is on prednisone or similar steroids, periodic bone scans should be done, perhaps semi-annually or annually.

Vasculitis patients with lung involvement will have imaging tests at extended intervals, perhaps semi-annual or annually, even in remission.

At least annual clinical examinations is prudent.

 ------->  IMPORTANT  <---------
AV patients will require lifelong follow-up testing to guard involvements in organs that may be symptom free.

Once in remission, AV patients can and should use inexpensive dipsticks to test urine for blood and protein at least every two weeks.

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II - DIAGNOSTIC PROCEDURES:   Presenting and past symptoms and family medical histories; these are the clues which the physician finds in a clinical exam, or as revealed in medical history or by the patient.  Endoscopic examinations may also reveal tissue abnormalities. AVs are diagnosed by the following methods:

    •    Blood chemistry tests and cell counts.

    •    Analysis of urine, both chemistry and microscopic.

    •    Imaging tests such as x-ray, MRI, and CT scan, which show lung infiltrates or nodules typical of granulomatosis or vascular
         tissue damage.  A CT virtual bronchoscopy can help identify stenosis and larynx problems.  Digital subtraction 
         angiograpy (DSA) may be useful in identifying stenosis or other abnormalities caused by vasculitis.

    •    Biopsy of the suspected granuloma and/or blood vessels or other affected tissues.  A positive biopsy for an AV in
         combination with symptoms and examination results is considered conclusive.

    •    Biopsies may be required of tissues where there is persistent inflammation problems in one or more of the following organs,
          as listed below:

                o    Sinus, nose, ear, throat, lung, joints, fever, kidney, trachea, eye, skin, peripheral nerves, inflammation of the heart
                       lining, central nervous system, and very rarely heart, pancreas, spleen, prostate, liver, testicles.
                o   
For WG only, the frequency of organ involvement is that of the preceeding parargraph.

    •    Other tests as required.  One might be measuring the blood flow in extremities to detect vascular stenosis.
   

    •   
Anyone with early emphysema or emphysema out of proportion to smoking history or unexplained liver disease should be
         screened for alpha-1 anti-trypsin deficiency (AATD).
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III - BLOOD TESTS AND COUNTS

NOTE THAT EACH MEDICAL LABORATORY ESTABLISHES THEIR OWN NORMAL RANGES FOR SOME TESTS WHICH MAY BE DIFFERENT THAN THE VALUES BELOW.

It is important to know the level of inflammation of the blood vessel walls.  Inflammation can be from many causes, other than an autoimmune vasculitis alone.  Two broad measurements of inflammations are the Erythrocyte (Red cell) Sedimentation Rate (ESR or SED), and the C Reactive Protein (CRP).  An abnormally high level of either can indicate inflammation of some type is present, but not what nor where, nor the cause

  A - TYPICAL NORMAL ESR VALUES *  **
 

 
AGE
NORMAL RANGE
Women
Less than 50
Less than 20 millimeters/hour
 
50 and over
Less than 30 millimeters/hour
Men
Less than 50
Less than 15 millimeters/hour
 
50 and over
Less than 20 millimeters/hour

     * Obviously there are some variations with age, so no precise numbers are true in all cases.

  **Some labs use these formulas: [(age divided by 2)=normal ESR] for males and 
[(age+10) divided by 2=normal ESR] for women.

  B - CRP
NORMAL LEVELS:  Less than 0.8 milligrams per
deciliter.  The normal level may also vary with age.  There
is now a high-sensitivity CRP for which results are reported as the
normal CRP, excepting multiplied by ten.  That is, the usual CRP
might be 0.7, but the h-s CRP would show 7.0.  

  C - BLOOD CHEMISTRY TESTS:  At the start of the diagnostic process, usually a blood chemistry panel is done to help screen out other diseases that might be causing the symptoms as well as to find what effects the disease has already caused.  At one time, chem  7 or chem 20 test panels were run on patients.  Now it's more common to order organ specific test panels that are both more pertinent and less costly.  For some autoimmune vasculitides, low Hemoglobin, elevated Alkaline Phosphatase and Platelets may correlate with vasculitis activity.

  D - BLOOD COUNTS:  Complete blood counts are also done.  For diagnosing an AV, probably the counts of various white cells is most significant.  Neutrophils are polymorphonuclear leukocytes, one of the several types of white blood cells (WBC).  See the table at the above link for information on blood counts.  It is to be expected the neutrophil counts will be high if WG is active and untreated.  Hign wosinophils counts are associated with Churg-Strauss syndrome, and perhaps some other AVs.

TYPICAL ADULT NEUTROPHIL NORMAL LEVELS

3.0 to 6.5 trillion per liter
(Usually expressed as 3.0-6.5 times ten to the ninth power, or simply 3.0 - 6.5 with the trillion assumed)

  E - ANCA TEST: 


IMMUNOFLUORESCENCE PATTERN ANCA TARGET ANTIGEN ASSOCIATED DISEASE
C-ANCA PR3 
WG
P-ANCA MPO MPA, CSS
P-ANCA HLE UC, CD, PSC, SLE
P-ANCA α-enolase
P-ANCA catalase
P-ANCA azurodicin (AZ)
 P-ANCA (atypical) lactoferrin (LF) UC, CD, PSC
P-ANCA (atypical) cathepsin G (CG) UC, CD, PSC, SLE, RA
P-ANCA (atypical) lysozyme (LZ) UC, CD, PSC
C-ANCA, P-ANCA (atypical) actin AIH
C-ANCA, P-ANCA (atypical) BPI UC, CD, PSC, AIH, SLE
P-ANCA (atypical) HMG1/2 UC, SLE, RA
P-ANCA (atypical) lamine B1 UC, CD, SLE
P-ANCA (atypical) histone H1 UC
 P-ANCA (atypical) 50 Kd UC, AIH

WG-Wegener's granulomatosis; MPA-microscopic polyangiitis; CSS-Churg-Strauss syndrome;
UC-ulcerative colitis; CD-Crohn's disease; PSC-primary sclerosing cholangitis;
SLE-systemic lupus erythematosus; RA-rheumatoid arthritis; AIH-autoimmune hepatitis; HLE-human leukocyte elastase;
BPI-bacteridical/permeability-increasing protein; HMG1/2-high mobility group of non-histone chromosomal proteins 1 and 2
  F - BLOOD TESTS:   Enzyme Linked ImmunoSorbent Assays   G - OTHER POSSIBLE BLOOD TESTS - Depending on physician's judgment.
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IV -URINE TESTS

  A - URINE CHEMISTRY TESTS

NORMAL CREATININE CLEARANCE VALUES


Male
Age 20 to 5
18.5 to 25.0 mg/kg/day (milligrams/kilograms/day)
 
Age 50 to 70
15.7 to 20.2 mg/kg/day (milligrams/kilograms/day)

 
Range:  0.8 to1.8 grams per 24 hours
Female
Age 20 to 50
16.5 to 22.4 mg/kg/day (milligrams/kilograms/day)
 
Age 50 to 70
11.8 to 16.0 mg/kg/day (milligrams/kilograms/day)
 
 
Range:  0.6 to 1.6 grams per 24 hours
 
Blood Component
Normal Values ##
Nitrite
Negative
PH (Acid/Alkaline)
5.0 to 8.0
Protein #
Negative
Glucose
Normal or negative
Ketones
Negative
Urobilinogen
Normal or negative
Bili Qual
Negative
Blood
Negative
Red Blood Cells
Less than 1 per HPF
White Blood Cells
2 per HPF

##  Measured values can vary from normal due to disease, medications, or medication interactions.

  B - URINE MICROSCOPIC TESTS:  Urine is checked for the following: V - IMAGING TESTS:   Imaging tests will be used to assess organ damage and reveal the characteristic granuloma of Wegener's as well as lung infiltrates or nodules.
VI - BIOPSY: A biopsy is a small tissue sample that is examined by a pathologist for abnormalities.
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VII - OTHER TESTS
VIII - SOME LINKS TO AV-RELATED MEDICAL TEST INFORMATION

    ANCA Interpretation
    Frequency Of cANCA, pANCA, Anti-MPO & Anti-PR3 In Vasculitis
    http://www.rdlinc.com/ancachrt.htm

   ANCA Tests:
    Anti-neutrophil cytoplasmic antibodies:
    Current diagnostic potential - 1996
    http://medicine.wustl.edu/~labmed/1996vol4no1.html

   Autoantibody tests:
    Auto-Antibody Patterns vs. Vasculitis- Table - Oct. 2001
    http://www.haps.nsw.gov.au/edrsrch/edinfo/autopat.html

   Blood Test Tables and Interpretations - America-On-Line
    http://members.aol.com/CatsPajama/bloodval.htm

  Complete Blood Count (CBC):
    http://www.healthwise.org/kbase/topic/medtest/hw4260/results.htm

  ELISA (Enzyme-Linked Immunosorbent Assay)
    http://www.uq.edu.au/vdu/ELISA.htm
    http://www.focusonthyroid.com/script/main/art.asp?articlekey=9105&rd=1

  How To Read Laboratory Tests
    Includes table of normal values for blood tests
    http://ighawaii.com/naturally/newsletter/lab.html

  How to convert U.S. concentration levels to the Syst'eme Internationale (SI)
    http://dwjay.tripod.com/conversion.html

  Imaging Tests for WG - Scroll down to Sections 4-9
    Thoracic Imaging of Wegener's Granulomatosis
    http://www.emedicine.com/radio/topic743.htm

 Immunology: Ref. Chart of Tests/Panels - [no longer available]
    Univ. of Washington, Dep't.. of Laboratory Medicine, Immunology Division
    Excellent summary of tests, updated in June 2000
    http://depts.washington.edu/labweb/dept/div/imm/refchart.html

  Lab Tests On-Line:
    Wonderful World of Tests - Links to various on-line lab test information
    http://www.enetis.net/~rbreske/lab.htm

  Lab Tests - basic
    http://www.tfn.net/healthgazette/labwork2.html

  Rheumatological tests:

  Rheumatology Laboratory Tests - Last update 1997
    http://www.rheumatology.org/publications/primarycare/number6/hrh0034698.html

 Systemic Rheumatic Diseases - Laboratory Testing
    http://www.postgradmed.com/issues/1998/02_98/ward.htm

 Urinalysis:
   Slides of Microscopic Examination
    http://www.lhsc.on.ca/lab/renal/

 Urinalysis: Informal but expert dissertation - Scroll way down for Urinalysis
    http://www.pathguy.com/lectures/urine.htm

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