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.
- The five
tested were: BVAS(BVAS/WG, BVAS2003),
a physician global assessment (PGA), theDisease Extent Index
(DEI), and the Five Factor Score (FFS).
- A patient with an autoimmune vasculitis
should ask the treating physician for one or more of the assessment
measures.
This web
page has eight sections.
To go to
any desired section, click on the section title below.
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.
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.
(Click
here to return to index)
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).
- All new or suspected vasculitis
patients
with lung symptoms should be screened for alpha-1 antitrypsin
deficiency (AATD).
- Click here
to view a web page about
alpha-1 antitrypsin deficiency.
- Since identification of the
hepatitis C virus (HCV) in 1989, it has become clear that the chronic
infection can be accompanied by a variety of systemic autoreactive
phenomena, most notably vasculitis [Feb. 2007 article].
- All known or suspected
autoimmune vasculitis patients should perhaps be tested for hepatitis
C.
- This virus
replicates very rapidly, turning over billions of times each day and
chronically stimulating the immune system.
- In
approximately half of patients, this chronic immune stimulation leads
to the development of autoreactivity and the appearance of rheumatoid
factor (RF) in serum.
- Some patients also are found
to have cryoglobulins, and a subset develop autoimmune conditions such
as cryoglobulinemic vasculitis with symptoms of purpura,
glomerulonephritis, peripheral neuropathy, and polyarthritis. B-cell
lymphomas also can occur.
- A new (2006) non-invasive test for
kidney disease, developed by clinicians at
Hammersmith Hospitals NHS Trust and Imperial College London, is
providing a simple, safe, cheap and reliable method of detecting kidney
disease.
- This new test
can accurately measure response to treatment, allowing clinicians to
tailor treatments to individual patients
- The test can tell us if a
treatment is working, and shows us, before it
is too late, if it's necessary to change the medication,
without the need to perform a biopsy.
- The test works by identifying
the amount of a cytokine molecule called monocyte chemoattractant
protein-1 (MCP-1) present in the urine.
MCP-1 is produced by the body as a response to inflammation, and
attracts white blood cells to the area to combat
pathogens.
-
A Feb. 2006 study noted that markedly increased numbers of
circulating endothelial cells (CECs) discriminate active vasculitis
from limited granulomatous disease and remission.
- A cut off value
of 20 cells/ml for a positive result yielded 64% specificity and 95%
sensitivity for active systemic vasculitis; the positive predictive
value was 63% and the negative predictive value 95%.
- These
findings add further proof to the concept of CECs as a marker of ANCA
associated small vessel vasculitis.
- A more recent study found the
level of CECs did not track the disease activity.
- Given the wide variety of organs and
tissues that can be affected by AVs, clinical examination alone is unlikely to result in a conclusive
diagnosis of an
AV.
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:
- The
Anti-Neutrophil Cytoplasmic Antibody
test)
- This test is somewhat specific for certain
autoimmune diseases affecting blood
vessels.
- It is done by separating out
neutrophils (one type of
white blood cell) and
staining them with a dye
that will fluoresce when exposed to ultra-violet light.
- The
illuminatedneutrophils are then
examined by
microscope to determine the character of the fluorescing granules in the
neutrophils.
- There are four recognized ANCA
immunofluorescence patterns:
- C-ANCA - classical cytoplasmic: A coarse granular cytoplasmic
fluorescence with accentuation between the nuclear lobes.
- Atypical C-ANCA: A
diffuse (flatter) cytoplasmic fluorescence without accentuation of the
interlobular fluorescence or glanular staining.
- P-ANCA- perinuclear: A typical perinuclear fluorescence with some
nuclear extension and granular cytoplasmic fluorescence on
formalin-fixed neutrophils.
- Atypical"
P-ANCA and atypical ANCA which include all other IIF reactivity: Pronounced
nuclear rim fluorescence with the center of nucleus unstained and
non-reactivity with formalin-fixed neutrophils, most commonly a
combination of cytoplasmic and perinuclear fluorescence.
- Points to consider about ANCA:
- The upper limit of normal cANCA
ratio varies from lab to lab. The upper normal limit for the test
result to be considered a“ negatives may be about 1:16 or 1:32.
- The cANCA test is about 90+%
accurate for WG for serious cases, but only 50% or so for light
cases).
Not all medical labs do the test, so frequently it is a 'send-out'
test.
A tentative diagnosis made by positive cANCA is often confirmed by
biopsy.
- The related pANCA test if
positive may be an indicator for some other autoimmune diseases, e.g.,
microscopic polyarteritis (MPA), Churg-Strauss syndrome (CSS),
polyarteritis nodosa (PAN) or
crescentic glomerulonephritis.
- In the case of mixed ANCA, it
may require other tests and analysis of symptoms to determine if the
cause of the abnormality is Wegener's or some other condition.
- Note that the cANCA and pANCA
are immunofluorescent (IIF) tests requiring a human microscopic
examination and interpretation. Results may vary if different
individuals are doing the test. It is best to have these and
other tests done at the same lab each time to reduce the possible
variations.
- While a positive cANCA is rather
diagnostic for Wegener's, some small percentage of Wegener's patients
test pANCA positive, rather than cANCA. Occasionally a Wegener's
patient will switch at times from testing positive to one ANCA to the
other.
- ANCAs are titrated tests.
Stained blood samples are diluted in steps, progressively until no
fluorescence is detected. Results are reported as the dilution
level at which fluorescence ceases. These are given as 1:256 or
1/256 for example. The higher
the ANCA ratio, the more of the harmful antibodies are present in the
patient’s blood.
- ANCA test results can be
positive due to conditions other than autoimmune diseases.
Amebiasis, ulcerative colitis, mesangiocapillary
glomerulonephritis with crescents, improperly cleaned
glassware, and both subacute and bacterial endocarditis
have been shown
to cause transient positive ANCAs.
- The table below shows
nneutrophil cytoplasmic antibodies
(ANCA) staining patterns and associated target antigen in patients with
systemic vasculitides and nonvasculitic disorders
| 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
- ANCA test samples can show
differing results if submitted to different labs. An effort to
standardize these tests is underway in Europe by the EUVAS group.
False positives or negatives can and do occur due to mishandling or
misinterpretation or even effects of medications or other disease
conditions.
- In diagnosis both cANCA by
immunofluorescence and anti-PR-3 by ELISA (see below) result in greater
sensitivity than either test alone, and are both are performed
in some labs even if only the
cANCA is ordered.
F - BLOOD
TESTS: Enzyme
Linked ImmunoSorbent Assays
- ELISAs use particular biological
proteins to detect specific biological markers of diseases.
- The
anti-PR-3 test relates generally to cANCA. The anti-MPO test
relates loosely to pANCA.
- Anti-PR3
ranges in use in the Mayo Rochester lab are:
- Less
than 6.0 EU/mL - negative
- 6.0 to
9.0 EU/mL - ambiguous
- greater
than 9.0 - positive
- Anti-MPO ranges in use in the Mayo Rochester
lab are:
- Less than 5.0 - negative
- 5.1 to 14.9 - equivocal
- above 14.9 - postive
- An ELISA test for anti-MPO
antibodies can be indicative of several autoimmune diseases such as
microscopic polyarteritis (MPA), polyarteritis nodosa, idiopathic
necrotizing and crescentic
glomerulonephritis as well as in periarteritis with glomerulonephritis.
- The newer Capture [Sandwich] ELISA
Test" can
be more sensitive in detecting the less usual forms of anti-PR-3.
In particular, the Capture ELISA using the MoAb 12.8 antibody was found
to have 96% sensitivity for WG in one study.
- The Immunoblot method is more
sensitive to
anti-PR3 than IIF, ELISA, or capture ELISA. It does not quantify
the
results which must be done by IIF, ELISA, or capture ELISA.
G - OTHER
POSSIBLE BLOOD TESTS - Depending on physician's judgment.
- It is probably prudent to
have an anti-glomerular basement membrane (anti-GBM) test done to
assure a seoond autoimmune disease isn't present that could threaten
kidneys.
- A November 2006 study of
autoimmune vasculitis ANCA postive patients found that plasma levels of
sIL2R, sCD30 and BAFF were higher in patients than in controls at all
time points.
- "Plasma levels of soluble
interleukin 2 receptor, soluble CD30, interleukin 10 and B cell
activator of the tumour necrosis factor family during follow-up in
vasculitis associated with proteinase 3-antineutrophil cytoplasmic
antibodies: associations with disease activity and relapse", and, "Plasma levels of sIL2R, sCD30 and IL-10
were higher at diagnosis and relapse than during remission."
- Testing of those proteins and
others may be commercialized at some time and be useful for determining
AV activity.
- There are some indications that
cytokines sICAM-1 and CCR5 may correlate with WG
activity. Tests for these might prove useful though
experience and
proof are lacking.
- As vasculitis patients often have silent (asymptomatic) heart
damage, it is good that they have their heart function evaluated by
electrocardiogram..
- A 2006 study showed that a serum protein, N-terminal pro-brain natriueuretic peptide
(NT-proBNP), can indicate the degree of heart
damage. The body
releases it when the heart's in trouble. It can not only diagnose the
disease, it also correlates with the magnitude of heart failure,
- There are some indications that
cytokines sICAM-1, interleukin-6 (IL-6), and CCR5 may correlate with WG
activity. These tests may prove valuable, though experience and
proof are lacking.
- It has been suggested that
anti-cardiolipin antibodies be tested for when ANCA test return a
positive result. Anti-phospholipid antibodies might also be
checked. These tests are not diagnostic for Wegener's, but may
uncover other autoimmune dysfunctions.
- Anti-Endothelial Cell Antibodies
(AECA) are found in the blood of 55-80% of Wegener's patients, and may
be involved in the etiology of WG though that isn't proven.
- Possibly related, a 2003 paper stated that it had
been found that soluble Endothelial protein C receptor (sEPCR) levels
in the plasma of patients with WG was shown to correlate with
disease activity and to rise prior to clinical relapse.
- There
are some indications that
the Procalcitonin (PCT) level may be a useful marker both for the
differential diagnosis between bacterial infection and inflammation,
but this remains unproven.
- ECP is a blood test for
eosinophilic cationic protein. These cationic proteins are among the
toxic granules that eosinophils release when they are activated. In
eosinophilic disease.
- The eosinophils infiltrate an
organ, inflame it, and the eos degranulate releasing these toxic
byproducts of eosinophils.
- These proteins are what
actually causes the tissue damage.
- So by testing for ECP, a
physician can tell more of the activity level of eosinophils.
- ECP is currently the
best blood test we have to look for active eosinophilic disease.
- Sometimes the regular eos
count on cbc is normal, but the ECP is high showing they are still
active.
- ECP is the best test to
reflect active Eosinophilia orf Churg-Strauss syndrome.
- It usually has to be sent
off to an out of state lab and takes about 2 weeks to come back.
- Pentraxin 3 (PTX3) level is
related
to vascular inflammation. Testing for PTX3 levels might be useful
in
determining vasculitis activity levels.
- A Jan. 2008 paper discusses the possible value of testing for the
Cystatin C level as it is more sensitive than the calculated eGFR and
so can reveal degradation of kidney function before the estimated glomerular filtration rate
(GFR) which is based on the 24 hr. creatinine clearance test..
IV -URINE TESTS
A -
URINE CHEMISTRY TESTS
- In remission, dipsticks should be
used bi-weekly to check for for blood and/or protein indicating silent
kidney involvement.
- Creatinine levels above normal are
likely to be evidence of kidney involvement. (NOTE: It is
possible to have creatinine levels within the normal range even while
kidneys are spilling blood and portein due to WG damage.)
- Urine creatinine can be tested
from a single sample.
- Single measurement normal range is
0.4 to 1.5 milligrams per deciliter (mg/dl)
- The more exacting test is the 24
Hour Creatinine Excretion (Clearance) test. The kilograms refers
to body weight. To convert pounds to kilograms, divide by 2.2.
- Renal function should be
assessed by the GFR using estimating equations
like the MDRD or Cockroft-Gault formula.
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
|
- Low Urine Creatinine:
- Values above decrease with
advancing age.
- Low value suggests inadequately
collected specimen
- Indicates probable kidney
damage due to WG or another cause.
- Urine chemistry is checked for:
|
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:
- Cell abnormalities
- White blood cells
- Red blood cells
- Transitional epithelial cells
- Squamous epithelial cells
- Renal tubular epithelial cells
- Fat Globules and Oval Fat Bodies
- Microorganisms
- Yeast
- Bacteria
- Trichomonas vaginalis
- Clinically Significant Casts
- Tamm-Horsfall protein
- Crystals (Most crystals
are not clinically significant)
- Crystals may be counted if
abnormal.
- Abnormal crystals may indicate
pH problems
- Miscellaneous Elements and
Artifacts
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.
- X-rays, Computed Tomography
(CT)
scans, and Magnetic Resonance Imaging (MRI) test may be done for sinus,
lungs, head, kidneys and
others as required. Color duplex
ultrasound (CDU) might prove useful in some cases to identify blood
vessel abnormalities. MRIs of sinuses are frequently false
positives as small amounts of mucus may appear as inflammation.
- F-18-fluorodeoxyglucose
(FDG) accumulates in inflammatory cells due to an increased metabolic
rate. FDG positron emission tomography (PET) represents a
proven imaging technique in patients with vasculitis.
FDG PET is a
useful tool for
evaluating the extent of vasculitis and evaluating the effects of
treatment of vasculitis. A recent development of a combined FDG
PET and CT uses 18F FDG to better locate and assess disease activity.
-
CT scans are generally preferable
to x-rays because of their considerably greater detail and diagnostic
utility, and are not significantly more expensive than x-rays.
There is greater radiation exposure so the number of CT scans should be
only those required.
-
MRIs may be done for soft
tissues
where x-rays and CT scans are not always useful. MRIs are done
in small tubes into which the patient slides and must remain
motionless. Larger machines less likely to cause claustraphobia
are available in some locations.
- MRIs done with contrast may use
gadolinium (gadodiamide). The gadodiamide agent poses less risks
the the odinated contrast agents formerly used. Poor kidney function probably precludes that
particular contrast medium. A new form of gadolinium encased in
carbon buckey balls may prove less toxic than gadodismide.
-
Tranquillizers or hypnotics may be
required for those with claustrophobia. Newer, larger MRI machines may
be available where claustrophobia may not be a problem.
- MRA (magnetic resonance angiogram)
is
particularly useful in identifying large vessel stenoses in the
brain.
- DSA
(digital subtraction angiography) may be useful in identifying and
monitoring
smaller blood vessel abnormalities.
- Recently a study showed diffusion-weighted
echoplanar MR imaging may be especially useful in diagnosing vasculitis
involvement in the brain (Central Nervous System).
- Videostroboscopy
may be useful in imaging the trachea and glottus.
- Early diagnosis of Takayasu's arteritis (and perhaps other
vasculitides) can be helped by use of 18F-fluorodeoxyglucose
positron emission tomography (18F-FDG-PET)
instead of the more common imaging tests.
- Virtual vascular endoscopy (VVE) uses two- and three-dimensional
(3D) data sets from magnetic resonance (MR) or computed
tomographic angiography to create endoluminal views of
blood vessels.
- A protocol of high-resolution magnetic
resonance imaging at 3 T in combination with contrast-enhanced magnetic
resonance angiography can help determine blood vessel abnormalities.
- Doing a "tagged white cell
count" (aka "leukocyte count") may identify areas of
inflammation. The test may be less invasive than the angiogram or
the arteriogram that use larger amounts of radioactive material.
For information, see
http://www.nlm.nih.gov/medlineplus/ency/article/003834.htm
VI - BIOPSY: A
biopsy is
a small tissue sample that is examined by a pathologist for
abnormalities.
- Some things to be aware of:
- Biopsies are usually done under
local anesthetic, excepting for open lung biopsies which require a
general anesthesia.
- Negative sinus biopsies are
notoriously unreliable because it is such a problem to get the correct
tissue sampled.
- Kidney biopsies are generally
rather trouble free and involve minimal intrusiveness if done by the
guided-needle method.
- Lung biopsies may be either by
guided-needle or an open-lung surgical method. The open lung is
major surgery and probably to be avoided if possible.
- Some physicians insist on an
open lung biopsy. It might be best to get a second opinion on the
necessity for that method before proceeding.
- More than once, a lung granuloma
as been incorrectly identified as lung cancer. It may be prudent
to
have the tissue sample submitted to a different pathologist in a
different hospital for evaluation.
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VII - OTHER
TESTS
- All
patients suspected of having vasculitis with lung or liver involvement
should be tested for alpha-1
antitrypsin antibodies (AATD).
- It is
prudent to check all vasculitis
patients for hypothyroidism (Hashimoto's thyroiditis)
or other malfunctions of thyroid and associated endocrine glands
(pituitary or hypothalamus).
- Because AVs can have clinically
silent cardiac involvement in about 3 out of 10 patients,
echocardiography is appropriate at times. As AVs can involve most any
organ,
there may be other tests required to identify those affected organs.
- Heart function should be systematically assessed in vasculitis
patients, with at least ECG and echocardiography, and more invasive
exploratory procedures when the former reveal abnormalities or symptoms
become manifest.
- Before starting therapy with
Imuran (Azathioprine), liver functions should be tested.
- Routine monitoring of patients
taking immunosuppressant drugs
- Monitoring of immune system
- Acute phase reactants
- C-reactive protein
- Erythrocyte sedimentation
rate
- Disease-specific
auto-antibodies
- Antineutrophil
cytoplasmic antibody
- Anti-double-stranded DNA
antibody
- Immunoglobulin and
complement concentrations
- Organ function and histology
- Neutrophil and lymphocyte
counts
- T-cell subsets
- Monitoring of adverse effects
- Haemoglobin
- Platelets
- Lipids
- Blood glucose
- Blood pressure
- Skin cancer surveillance
- Rectal examination
- Pap smear
- Possibly prostate specific
antigen
- Bone densitometry
- Cataract screening
- Therapeutic drug monitoring
- Meeting therapeutic targets
(where known)
- Defines poor absorption
- Helps assess compliance
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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