WEGENER'S GRANULOMATOSIS
A RARE AUTOIMMUNE VASCULITIS - (Compiled by a WG Patient)
Updated – 29 January 2003
This file has been superceded by many changes.
See mail.chartermi.net/~blader for most recent version
SECTION
TITLE
SECTION
1 - Sources For This Document
2 - Questions - Refer To Your Physician
3 - Feeling All Alone? Contact others
4 - Early Signs of WG
5 - Is There Life After WG? (Prognosis)
6 - You Need An Advocate
7 - Records You Need
8 - Causes of WG
9 - Description of WG
10 - Disease Features
11 - Symptoms-Percent of WG patients Table 1
12 - Symptoms after Remission Table 2
13 - Disease-Related Permanent Morbidity
Table 3
14 - Finding A Physician
15 - Talking To Your Physician
16 - Conventional Treatment
17 - Medication Dosages
18 - Medication Side Effects
TITLE
19 - Alternative Corticosteroids
20 - Alternative Immunosuppressives
- Table 4
21 - When Taking Immunosuppressives
22 - Poly- and Mono- clonal Antibodies
23 - Newer Monoclonal Antibodies
Under Development - Table 5
24 - Newer Drugs Under Development
25 - Rescue Therapies
26 - Osteoporosis & Peripheral
Neuropathy
27 - Ulcerations
28 - Life-long Follow-Up
29 - Subglottal Stenosis- Table 6
30 - Nasal Irrigation
31 - Diagnostic Tests
32 - Similar Diseases - Table 7
33 - National Institutes of Health
34 - Reference to Cytoxan & Prednisone
Side Effects
35 - Reference Web Pages (URL's)
36 - Medical Tests for Wegener’s
To download an printable copy of this page in Microsoft Word (.doc) format, click here.
Para descargar una versión EN ESPAÑOL en la palabra de Microsoft (doc) ajuste a formato, chasque
aquí.
To download an M/S Word printable copy of the
Index of the 10th International Vasculitis and ANCA Workshop
held Apr. 25-28, 2002 Cleveland, Ohio, click here
Page 1 of 23
1 - SOURCES: The following information is derived from a variety of sources over more
than five years and is not to be considered as medical advice, but merely the opinions or
experiences or findings of the writer who is not a physician and is not medically trained.
Much comes from Medline abstracts and medical journal articles. Some is from WG
patients and carers, some from newsgroups, Internet web pages, etc.
The writer does not vouch for the accuracy, completeness, nor applicability of the
information below to any person, whether a WG patient or otherwise. No medical
decisions should be made on the basis of information on this web page.
2 - QUESTIONS: ALL MEDICAL QUESTIONS, SYMPTOMS, CONCERNS AND
PROBLEMS SHOULD BE DIRECTED TO LICENSED MEDICAL PROFESSIONALS.
3 - FEELING ALL ALONE? You can contact the Wegener's Granulomatosis Association
(WGA) in Kansas City to request a "Patient Packet" with good information on the disease
and treatments. In addition, you can request a "Physician's Packet" in case your physicians
aren't familiar with WG.
Wegener's Granulomatosis Association (WGA)
P. O. Box 28660
Kansas City, Missouri 64188-8660
Toll free (in U.S.) 1-800-277-9474,
Fax & phone (foreign) 1-816-848-4444
E-mail to mailto:wga@wgassociation.org
The WGA web page is at http://www.wgassociation.org
The patient's packet will have a list of WGA members in your state or province or country
with names, cities, and phone numbers all listed so you can contact someone close to you
who is familiar with WG.
The WGA puts out a bimonthly newsletter for an annual due of U.S. $20 ($25 outside U.S.).
(Tax-deductible donations gratefully received). Each newsletter carries a list of contact
persons for various locations. The WGA is donor and member supported. Please help
keep this invaluable organization funded.
A WG E-mail group has 400+ subscribers. The patient's and caregivers there have lots of
information, experience, and most importantly, support. Information is at:
http://www.weareb.org/mailman/listinfo/wg-discussion
There is an on-line chat (IRC) on Monday evenings at 9 PM ED/ST, in the "wegener"
channel on NewNet. Use an IRC client program such as mIRC (PC) or IRCLE (Macintosh)
to connect. Note the channel is NOT "wegener's", nor "wegeners", but merely "wegener"
(without the "").
A bi-annual symposium is held every even-numbered year in Kansas City. Attendance is
limited to about 400, so hotel and attendance must be reserved early. The next will be the
in October 2004, and probably in Kansas City, MO. Hotel reservations must be made
separately from Symposium registration. The Symposium is an excellent way to meet many
WG patients and to learn from the many presentations by leading researchers and
clinicians. Information will be at
http://www.wgassociation.org/aboutwga/symposium.shtml
Page 2 of 23
4 - EARLY SIGNS: Persons with persistent sinus/nose/ear/throat problems that do not
respond to normal treatments should consider the possibility of Wegener's Granulomatosis
(WG). WG is an autoimmune vasculitis which can be a relatively slow moving disease, or
very sudden and severe. It can do irreversible damage in a short time, but often it smolders
before finally becoming acute.
WG can present many other initial symptoms in addition to the usual airway symptoms;
sinuses, throat, trachea, and lungs. Other symptoms might be excessive fatigue, night
sweats, joint pains and other arthritic-like symptoms. One fairly distinctive sign is "saddlenose deformity", where the bridge of the nose collapses due to cartilage destruction (though
there are other conditions that can cause the same symptom). [After remission, this
deformity can often be reconstructed (not "cosmetic surgery, but reconstruction)]
(See Section 9, "Description of WG" for more on symptoms)
(See Section 36, "Medical Tests and Wegener's" for more on diagnostic testing)
5 - IS THERE LIFE AFTER WG? (Prognosis): Many WG patients lead normal or nearly
normal lives. (See Section 10 below). The earlier the disease is diagnosed and treated
appropriately (usually that means "aggressively"), the more likely that there will be little longterm damage. Some WG patients with kidney damage recover enough function to be off
dialysis. Renal (kidney) function may return up until 6 months after start of therapy
especially if started soon enough (or perhaps even longer). Renal failure severe enough to
require dialysis during the acute phase of the disease does not preclude a good initial
response to therapy. Between 55 and 90 percent of these patients will recover enough
function to come off dialysis. The outcome in these patients may be enhanced by initial
plasmapheresis.
Relapse is not unusual. Factors increasing the likelihood of relapse include cumulative
Cytoxan less than 20 grams, steroid use less than four months, and initially presenting with
renal involvement. Furthermore, the risk for relapse is modulated by the presence or
absence of the staphylococcal tsst-1 gene.
6 - YOU NEED AN ADVOCATE: If possible, take an advocate with you to every
appointment to take notes, ask questions, and remember the details that later you have
already forgotten. When one is ill, he or she is hardly in a position to ask the right
questions, nor to understand nor remember the answers. The advocate should help you
prepare a list of questions before each appointment.
7 - RECORDS: Before you get involved in the process of treating any serious disease, the
first and most important thing is to buy a notebook and keep a journal. A journal will help
you track the disease symptoms and medication side effects that are otherwise easily
forgotten. Your advocate should help you prepare a list of questions for your next
appointments and keep the questions and answers in your journal.
Write down
EVERYTHING! Every symptom with date and time, every office visit, every test and test
result, and every medication and the effects that they have on you. Before every lab and
radiographic test, request the lab mail you the results directly. If you have one, use your
computer for record keeping, both text and spreadsheets.
Have your physicians mail you copies of your medical records after each visit, if they will do
so. In some states you own the record, in others it may be the physician's option, and you
may be charged for the cost of making copies. An M/S Word file on AARDA information on
diagnosis of autoimmune diseases can be downloaded from:
http://mail.chartermi.net/~blader/Diagnosis-AARDA.doc
Page 3 of 23
8 - CAUSES OF WG: The causes of WG are not known. The immune system produces too
much or the wrong type of antigens or cytokines that then cause neutrophils (white blood
cells) to attack the endothelial cells that form the lining of blood vessels. Autoantibody
repertoires from patients with PAN WG, CSS and MPA are disease specific.
Most WG patients seem to be able to identify a period of excessive physical or emotional
stress just prior to developing symptoms of WG or subsequent flare. There is some
indication that persons whose reaction to stress is higher heart output, rather than stiffening
of arteries may produce changes in the immune system.
Bacterial and/or viral infections appear to be involved in the development of the disease but
proof is still lacking. It has been demonstrated that patients with chronic colonization of the
nasal mucosa with Staphylococcus aureus (Golden Staph) are at an increased risk for
relapse. Recent work seems to implicate S. aureus in initial disease development.
In one study, a periodic fluctuation of the frequencies of WG with peaks every 3-4 years was
noted for patients with ANCA related vasculitis (WG, MPA, RLV) during the 21-year period
1975-95. Onset of symptoms was predominantly noticed during the winter months
(December-February in the northern hemisphere) for patients with a positive cANCA blood
test result.
The vascular infiltrates in WG are predominantly composed of T-lymphocytes and
macrophages.
There is increased incidence of mild to moderate a1-AT deficient
phenotypes in cANCA, but not pANCA-associated vasculitis.
Silicon, chlorated
hydrocarbons, drugs and infections are environmental factors relevant to the occurrence of
small vessel vasculitis.
Hepatitis C infection has been implicated in a number of vasculitis diseases. There may be
unwarranted concern about vaccinations.
Patients with connective tissue diseases (CTDs, which include WG)) treated with chronic
corticosteroids have increased risk factors for Obstructive Sleep Apnea Syndrome (OSAS)
due to weight gain and steroid affecting the respiratory and pharyngeal muscles. Patients
with the following symptoms warrant screening for exam findings possibly predictive of
OSAS including apnea witnessed by others, habitual loud snoring, daytime somnolence,
non-refreshing sleep, nocturnal restlessness, morning headache, increased neck
circumference and poor visualization of the posterior pharynx.
Polymorphisms in the CXCR2 gene are associated with WG, suggesting a role for IL8/CXCR2 interaction in WG. Production of IL-8, a potent chemokine for polymorphonuclear
neutrophils, is increased in WG.
9 - DESCRIPTION OF WG: WG is a systemic disease, a form of vasculitis which affects
mostly smaller blood vessels, down to capillary size, thus it can lead to cell death and organ
impairment or failure. It can strike at any age, and equally among both sexes (unlike many
autoimmune disease which have a higher percentage of female patients).
Recent
estimates of WG frequency vary from about 0.8 to 1.2 per 1,000,000. Older estimates of 35 per hundred thousand seem more likely to be true. It seems likely many cases of WG are
never correctly diagnosed and those frequencies may reflect a significant undercount.
Overlap with other AI vascular diseases may also cause reported counts to be lower than
actual. Causes of death may be categorized as kidney failure etc., rather than the
underlying cause of WG.
Page 4 of 23
(9 - DESCRIPTION OF WG, Cont’d.)
WG affects various organs with the approximate frequency listed (with most frequent first):
Sinus, nose, ear, lung, joints, kidney, trachea, eye, skin, peripheral nerves, central nervous
system, and very rarely heart, pancreas, prostate, liver, testicles. WG without kidney
involvement is known as "limited WG”. That does not mean it is less dangerous.
Vasculitis predisposes one to blood clotting so can occasionally cause stroke or stroke-like
symptoms. Any diminution in central nervous system function should be reported to one's
physician at the first opportunity.
(See "Sections 11 & 12 - "Table 1" and "Table 2" for more on symptoms)
10 - DISEASE FEATURES:
 WITH VERY RARE EXCEPTIONS, WG IS FATAL UNLESS TREATED.
 With early diagnosis and treatment, most patients lead a normal or nearly normal life.
 WG is neither contagious nor hereditary in so far as is known.
 There is a genetic component to developing WG.
 Although the disease remains incurable, there are several effective treatments.
 About 95% of WG patients are able to achieve remission that may last an
indeterminate amount of years or months.
 Average time from onset of acute symptoms to diagnosis is five months, but the
mean time is fifteen months (half less than 15 months, half more than 15 months)
 WG can occur at any age. WG has its peak in a person's 40s and 50s
 The age range of patients is from 5-91 years. 85% of patients are above 19 years old.
The average age of patients with WG is 41
Out of all WG patients: 97% are Caucasian, 2% are Black, 1% are of another race
(These figures may represent under reporting in lower income persons)
Page 5 of 23
11 - PERCENT OF WG PATIENTS WITH SYMPTOMS:
NOTE - The data in the following tables is some years old. Newer studies might come up with different figures.
TABLE 1
Symptom
Ear, Nose, Throat
Lung
Joints
Fever
Kidney
Cough
Eye
Skin
Weight loss
Pericarditis
Peripheral nerves
Central Nervous System
Incidence At
Onset (%)
75
50
30
25
20
20
15
15
10
5
0
0
Total %
After Time
95
85
70
50
75
50
50
45
35
10
15
10
Some other rare organ involvements seem to include spleen, intestines, testicles, heart, and even
prostate. Patients with spleen involvement may have negative cANCA.
Page 6 of 23
12 - SYMPTOMS AFTER REMISSION - Remission is not well defined, but generally
means no progression of symptoms, relatively normal blood, urine, and radiographic
results while entirely off medication, or on low, maintenance dosages.
TABLE 2 (All ages)
Symptom
# Chronic renal disease
Hearing loss
## Cosmetic nasal deformity
Hoarseness and tracheal stenosis
Visual loss
Patients (%)
40
35
30
15
8
# Kidney transplant may be possible if less than 65 and in remission
## Can often be corrected surgically after WG is inactive.
Other symptoms may be chronic depending on disease severity at time of treatment.
13 - DISEASE-RELATED PERMANENT MORBIDITY
TABLE 3
Type of Morbidity
Chronic Sinus Disease
Renal Insufficiency
Hearing Loss
Nasal Deformity
Pulmonary Disease
End-stage Renal Disease
Subglottal Stenosis
Blindness
Childhood Onset
WG (%)
48
35
17
48
14
9
35
9
Adult Onset
WG (%)
47 (Note 2)
43 (Note 1)
38
25
18 (Note 2)
13
9
8
Note 1. The median serum creatinine for the eight childhood-onset patients and
58 adult-onset patients with renal insufficiency was 1.7 and 3.2 mg/dl (150 and
288mmol/L), respectively. Six adult-onset and two childhood-onset patients
received renal transplants.
Note 2. Medical treatment or surgical treatment or both may have also contributed
to permanent morbidity.
Page 7 of 23
14 - FINDING A PHYSICIAN: Most physicians have never diagnosed a case of WG. If you
suspect WG, you need to find a physician well experienced in diagnosing and treating
autoimmune vasculitides. A rheumatologist or internist is frequently a good choice,
although a nephrologist, ophthalmologist, and otolaryngologist can also be helpful. Many
WG patients have had numerous visits to ENTs and other specialists before being correctly
diagnosed.
Women especially are often told they are imagining or exaggerating their ailments. NOT
so! Keep looking until you get positive results. Ask to be on the cancellation waiting lists to
get earlier appointments. If in serious trouble, go to an Emergency Room and hope they
have the expertise needed. Mention you suspect autoimmune vasculitis.
Major medical centers and teaching hospitals are available if one can't locate the needed
care locally. It seems best to be seen by a "Fellow" or "Department Head", but not always.
An intern or resident may have more recent training that included the rare autoimmune
vasculitides. Two notable Vasculitis Centers are:
Cleveland Clinic Center for Vasculitis at
http://www.clevelandclinic.org/arthritis/vasculitis/default.htm
Johns Hopkins at:
http://vasculitis.med.jhu.edu/
Every new physician you will have to deal with will want a medical history, and there are
usually a number of physicians involved over time so keep those records up to date in your
files. If you don't get answers from your initial physician, get a new physician, and insist on
a copy of your previous medical records. Keep trying until you get good answers and
effective treatment. Have your physician contact one or more of the WG Association
consultants. See http://www.wgassociation.org/aboutwga/consultants.shtml
For information from the AARDA on getting a proper diagnosis, see:
http://pws.chartermi.net/~blader/Diagnosis-AARDA.doc.
For persons with no or limited medical insurance, the National Institutes of Health have ongoing clinical studies. If a WG patient qualifies, most medical expenses will be paid while in
the study. See section 33 - NATIONAL INSTITUTES OF HEALTH below.
15 - TALKING TO YOUR PHYSICIAN: See http://elfstrom.com/arthritis/appointments.html
It is best if one of the various physicians treating your WG will take the lead in coordinating
with your other physicians. Unfortunately, it is sometimes difficult to get one to do that.
Patients sometimes get bounced between specialists who don't talk to each other. In that
case you have to take an active role in keeping all your physicians informed.
You can't assume that your various physicians will communicate with each other. Request
each physician's office to copy each physician's report, sending copies to your other
physicians. Do the same with lab test reports. When it appears necessary, insist that they
confer.
Page 8 of 23
(15 - TALKING TO YOUR PHYSICIAN, Cont’d.)
Don't wait to notify your physician(s) of any new symptoms or changes in existing
symptoms.
Many physicians today have fax and/or e-mail facilities. Your physician may indicate that he
wants you to refer non-urgent messages by one of those rather than telephone. That way
you both will have a record of what was sent and received.
Always consult your physician before taking herbal or other non-prescription medications
and supplements.
Ask your physician about adjusting your medication levels according to age.
Problems with office staff should be referred directly to the physician and preferably in the
presence of an advocate.
16 - "CONVENTIONAL" TREATMENT:
Warning - Persons on corticosteroids should have their medical condition clearly noted in purse or
wallet. It is rather dangerous for a person on steroids to miss the required dosages.
Some WG patients find it sensible to register with MedicAlert where a single phone call by
emergency personnel can access the patient's medical history, diseases, and medications
before treating the patient. Emergency personnel are trained to look for bracelets or
necklaces with the needed contact information.
WARNING: Frequent measurements of blood cell counts must be made regardless of the
immunosuppressive used to treat the patient. This is to assure the patient doesn't become highly
susceptible to infection due to excessive suppression of lymphocytes (white blood cells).
"Standard" treatment originally was Cyclophosphamide (Cytoxan, an immunosuppressive)
and a corticosteroid (often Prednisone) for about one year after remission (the "Fauci"
regimen). The Prednisone is fairly fast acting, within a day or two. It may take 2-4 weeks
for the Cytoxan or Methotrexate to take effect.
WARNING: Quinine compounds such as Plaquenil increase the toxicity of Cytoxan. Persons on
Cytoxan should have their blood tested weekly to avoid over-suppression of the lymphocytes).
There are other immunosuppressives and corticosteroids available for patients who can't
tolerate Cytoxan or Prednisone. The effectiveness of daily oral versus periodic intravenous
administration of Cytoxan is a matter of medical discussion.
More recently, some use Cytoxan and Prednisone until the disease starts to abate, then
substitute Methotrexate or another alternative immunosuppressive for the Cytoxan until
remission or some time after. (It is important to monitor blood counts if using the
combination of Methotrexate and Bactrim, as this may lead to severe bone marrow
depression.) Methotrexate appears to be a folic acid antagonist, so some physicians
prescribe folic or folinic acid for patients on Methotrexate. Use of one of the various quinine
compounds may help alleviate the side effects of Methotrexate. Methotrexate dosages are
generally in the 15-25 mg/day of oral medication (or 0.6-1.0 mg IV).
Page 9 of 23
(16 - "CONVENTIONAL" TREATMENT, Cont’d.)
The Prednisone dosage is usually tapered very carefully, but as quickly as possible after the
initial acute phase to avoid the long-term side effects of the steroid. WARNING - NEVER
CHANGE CORTICOSTEROID DOSAGES SUDDENLY, BUT ONLY WITH THE KNOWLEDGE AND
CONCURRENCE OF YOUR PHYSICIAN. This is especially important below 10 mg/day of Prednisone.
Light cases of WG without serious organ involvement may use only Bactrim (Septra) and
Prednisone, though opinions on this treatment vary. Unusually a patient may go into
remission on Prednisone alone. And extremely rarely, a patient may go into remission
spontaneously.
Bactrim is used for prevention of pneumocystis carinii pneumonia (PCP), and perhaps for its
efficacy in suppressing WG by some unidentified mechanism. For persons allergic to
Bactrim, Mepron (Atovaquone) may be used to prevent PCP, but is probably not effective
against Stapylococcus aureus, the bacteria that has been identified as being associated
with relapses. An antibiotic effective for treating S. aureus may be the new Linezolid.
Pentamidine may also be considered in place of Bactrim. It is important to monitor blood
counts closely if using the combination of Methotrexate and Bactrim, as this may lead to severe bone
marrow depression.
For preventing relapse, low dose Methotrexate seems to be superior to Bactrim in terms of
efficacy, but has more side effects. Desensitization techniques can sometimes allow
persons normally allergic to sulfa drugs to take Bactrim.
A recent study indicates switching cyclophosphamide to azathioprine after 3 months of
remission in patients with PR3-ANCA associated vasculitis who are still ANCA positive at
the moment of treatment switch may result in increased possibility of relapse, although this
is considered unproven.
Sometimes Mesna is prescribed when using Cytoxan to help avoid bladder cystitis and
irritation. For that reason too, some physicians prescribe intravenous injection of Cytoxan
periodically, rather than daily oral doses. Moderate exercise can be an important part of
therapy as long as done with the physician's approval.
17 - MEDICATION DOSAGES
Physicians use their individual experienced judgments about the best medication dosages
for each patient, as well they should.
The usual starting dosage for oral Cytoxan seems to be 2 mg/kg/day (about one milligram
per pound of body weight) that is about 150 mg/day for a 165 lb. person. This is sometimes
doubled to 4 mg/kg/day for acutely ill patients.
Prednisone starting dosage is usually 1 mg/kg/day or about 75 mg for the same 165 lb.
person. Again, in acute, life-threatening cases, as much as 1000 mg/day might be given for
a brief time before tapering to a more normal starting dosage. Other corticosteroids will
perhaps require different dosages for the same results.
At low dosages, steroids must be tapered carefully to avoid serious life-threatening results.
Below 10 mg/day of Prednisone, a taper of 1 mg every 7-30 days is common. One mg
tablets of Prednisone are available for tapering slowly.
Page 10 of 23
(17 - MEDICATION DOSAGES, Cont’d.)
Some physicians will specify a taper using an "alternate day" method in which it is
attempted to get a dormant adrenal gland to start producing cortisone or to prevent adrenal
insufficiency.
WARNING - NEVER CHANGE CORTICOSTEROID DOSAGES SUDDENLY, BUT ONLY WITH THE
KNOWLEDGE AND CONCURRENCE OF YOUR PHYSICIAN. This is especially important below 10
mg/day of Prednisone.
18 - MEDICATION SIDE EFFECTS: Section 34 below links to information on side effects of
Prednisone and Cytoxan. Those listed are many, but most patients don't experience
anything approaching the complete range.
Virtually all medications have side effects that vary from person to person, so patients can
expect some side effects, regardless of medications used to treat their disease.
It is very difficult for patients and physicians to tell what particular symptoms are caused by
the disease, and those caused by medication side effects. One should keep one's
physician aware of changes in current symptoms, as well as new ones.
Osteonecrosis of the hip stands at 11% for long-term Prednisone use, with or without
supporting risk-reducing medication. The 11% comes from those who through blood tests
have been determined to have a missing Protein C, S, or Factor V Leiden in the blood. This
creates an inability to break down clots in the blood. This is a contributing factor to
Prednisone-based osteonecrosis.
Leukovorin (Tetrahydrofolic or Citrovorum Factor) that is a reduced form of folic acid is
sometimes needed as a MTX 'rescue' when MTX causes dangerous side effects.
WARNING - NEVER CHANGE CORTICOSTEROID DOSAGES SUDDENLY, BUT ONLY
WITH THE KNOWLEDGE AND CONCURRENCE OF YOUR PHYSICIAN. This is
especially important below 10 mg/day of Prednisone.
(See Section 34, Reference to Cytoxan and Prednisone side effects below)
19 - ALTERNATIVE CORTICOSTEROIDS: Some alternatives to Prednisone are
Methylprednisolone, Medrol or Dexamethasone. Both intravenous and oral forms are
available under a variety of trade names. Equivalent dosages vary among the various
drugs.
In Germany there is apparent use of intramuscular depot steroid every three weeks as
opposed to daily oral use. A new drug Deflazacort (Azacortid, Calcort, Lantadin) is under
study but apparently withdrawn. It was hoped the drug would have less side effects than
Prednisone, but a recent data puts that claim in question. It perhaps is being used outside
the U.S.
Page 11 of 23
20 - ALTERNATIVE IMMUNOSUPPRESSIVES:
TABLE 4
Drugs other than Cytoxan and Methotrexate
OTHER CYTOTOXIC IMMUNOSUPPRESSIVE DRUGS USED FOR WG IN SOME COUNTRIES
Cellcept /
Sandimmune / Cyclosporin /
Imuran / Azathioprine ##
Mcycophenolate Mofetil
Neoral / SangCya
# Arava / Leflunomide
Etoposide / VP-16 / VePesid
Leukeran / Chlorambucil
# The Federal Drug Administration has warned of liver damage using Arava
## TPMT Liver functions should be checked before starting Imuran. The test is TPMT Mutation Analysis.
TPMT stands for thiopurine S-methyltransferase and is the primary enzyme responsible for thiopurine
drug-based metabolism. (Imuran/azathioprine is a thiopurine drug). If a patient has low TPMT enzyme
activity they are at high risk for dosage-related side effects.
USED FOR OTHER AUTOIMMUNE DISEASES WITH POSSIBLE APPLICATIONS TO WG
Mercaptopurine / Purinethol
Tacrolimus / Prograf /
Sirolimus / Rapamycin (?)
FK-506B ###
Interleukin-10
Azulfidine (?)
### If Tacrolimus or Cyclosporine are used, a small percentage of WG patients will suffer kidney damage.
21 - WHEN TAKING IMMUNOSUPPRESSIVES: If you now or ever tested positive for
tuberculosis, then it is mandatory that you be treated with appropriate antibiotics for
a long course (6 months perhaps) to make sure your TB doesn't become active. If
you've never been tested for TB, ask your physician about a test before starting
immunosuppressives. The TB test cannot be done while taking Prednisone or other
corticosteroid.
Blood counts, particularly lymphocyte counts must be monitored closely, perhaps weekly
while on immunosuppressives to avoid infection caused by excessively low white cell
counts.
Anyone on immunosupression should be very careful about not receiving live vaccine
themselves, for example, polio, measles, mumps, rubella. Ask your physician before
receiving vaccinations to be sure the vaccine doesn't contain live viruses or bacteria.
With a suppressed immune system, one is susceptible to opportunistic infections. To avoid
infections, keep hands well washed frequently, avoid crowded in-door spaces and obviously
ill persons; keep hands from eyes, nose, mouth and face. Don't share eating utensils.
Avoid scrapes, cuts etc. Use an electric razor to avoid razor cuts. Wash all fruit and
vegetables. Don't eat under-cooked meat, fish, or eggs. Any dental work or invasive
procedures might require an antibiotic just before the appointment. Discuss with your
physician and dentist.
Bactrim (Septra) is often prescribed while one is on immunosuppressives to avoid
Pneumocystis Carnii Pneumonia (PCP) and other opportunistic infections. For patients
allergic to Bactrim, ATOVAQUONE (Mepron®) suspension may be effective. Valtrex can
perhaps be taken daily to avoid activating herpes zoster (cold sores, shingles). New studies
are ongoing to see if anti-staphylococcal nasal ointments have an efficacy similar to Bactrim
(Septra) in preventing relapse. S. aureus binds more easily to endothelial cells than most
bacteria.
Page 12 of 23
(21 - WHEN TAKING IMMUNOSUPPRESSIVES, Cont’d.)
Use of live-culture yogurt or capsules of lactobacillus (and sometimes other bacteria) to
replace G/I tract flora may help prevent digestive tract problems when on antibiotics. The
yeast Saccharomyces boulardii may help prevent getting a Clostridium difficile infection in
the G/I tract, which sometimes occurs while on antibiotics for an extended period.
Immunosuppressed patients can develop painful mouth and tongue sores that don't heal
well. Also Candida Albicans (Thrush) can be common. Physicians can prescribe a variety
of anti-fungals and other medications to help. For nausea and indigestion, Zofran or other
similar medications can be used. Various anti-virals are available for shingles but must be
used at first signs to be effective.
Persons taking Methotrexate or Bactrim (Septra) should discuss with their physician the
need for folic or folinic acid.
22 - POLY- & MONO-CLONAL ANTIBODIES USED FOR WG:
(Note - Not all used extensively).
Polyclonal rabbit anti-thymocyte globulin (ATG) has been reported to be effective in 4/5
patients with severe, refractory SV (Systemic Vasculitis) though it appears there are enough
risks to limit further research.
A family of drugs coming onto market that promises to be effective, but perhaps expensive
are monoclonal antibodies (ABs). The use of murine (mouse) derived antibodies is giving
way to humanized or human antibodies that are less likely to cause allergic reactions.
Newer drugs are being developed to target the particular cytokines that cause the immune
system malfunctions, rather than attacking a broad range of cells not involved in
inflammation.
OKT3 (Orthoclone) has been used to treat autoimmune vasculitis, but with serious side
effects. It is a murine monoclonal anti-lymphocyte antibody, which has recently been
humanized and modified so it doesn't bind to Fc receptors. The newer version is known as
Aglycosyl CD3 antibody. More testing is required prior to human use.
Treatment with one murine monoclonal AB, CAMPATH-1H used to deplete lymphocytes,
has been effective both in patients with primarily cell-mediated disease, and somewhat to
the researcher's surprise, it also worked very well in patients with autoantibodies, e.g.
Wegener's granulomatosis.
At least one Australian patient reports effective treatment with Campath in the U.K., though
it may have been an earlier version of Campath that was used in that case. Campath
(Alemtuzumab, anti-CD52) has been approved by the FDA for use in treating B-cell chronic
lymphocytic leukemia. Presumably, any U.S. physician could prescribe it if she/he deems it
advantageous and safe, and it may indeed be used for WG in the U.S. in the future.
It has been reported, "A regimen of humanized monoclonal anti-lymphocyte antibodies
(mAB) was used as successful intervention for intractable Wegener's granulomatosis.
(Exactly what drug was not specified in the source).
The combination of CAMPATH-1H followed by CD4 antibody has been able to induce
remission in some patients with vasculitis that had become resistant to CAMPATH-1H as
well as all conventional therapies.
Page 13 of 23
(22 - POLY- & MONO-CLONAL ANTIBODIES USED FOR WG, Cont’d.)
Trials of anti-CD18 antibody (LDP-01) antibody to treat autoimmune vasculitis and in kidney
transplantation have been carried out. Some success with treating vasculitis has been
reported.
Recently one case of refractory WG was put into remission by a combination of
glucocorticoids, and Rituximab (Mabthera, an anti-CD-20 monoclonal AB used to deplete Bcells).
15-Deoxysperbualin (DSG) has show to be effective in treating WG patients with refractory
disease.
23 - NEWER MONOCLONAL ANTIBODIES UNDER DEVELOPMENT: Monoclonal
anti-TNF (Tumor Necrosis Factor) drugs that may have been tried for WG with some
successes are:
Avakine = Remicade = Infliximab - (Anti-TNF in use for RA and other autoimmune diseases)
Enbrel = Etanercept - (Anti-TNF under study now in U.S. for use against WG. Anecdotal
info only - Results seem promising; Demyelinating disease noted a
side effect).
TABLE 5
MONOCLONAL ANTIBODIES (mABs) UNDER DEVELOPMENT WHICH MIGHT BE EFFECTIVE FOR WG
DRUG
MANUFACTURER
BMS-188667 (CTLA-4-Ig) for autoimmune diseases
Bristol-Myers Squibb
(In Ph. 1 & Ph 2 Clinical trials)
Anti-TNF (anti-Tumor Necrosis Factor) drugs:
D2E7 (Adalimumab)
CDP870
ISIS 104838 (An anti-sense TNF-alpha inhibitor)
J695 (neutralizes IL-12)
Abbott Laboratories
Celltech
ISIS Laboratories (Ph. II for R.A.)
Wyeth-Ayerst Laboratories
CAT192 (human anti-TGFß monoclonal AB)
Kineret (Anakinra, Anti-IL-1 ra monoclonal antibody)
For Systemic sclerosis
Amgen (approved for rheumatoid
CD4 antibody
CD18 antibody (LDP-01)
TolerRxInc
LeukoSite, Inc.
(Phase 1 Trials for Crohn's Disease)
Arthritis)
(affects white cell adhesion)
Aglycosyl CD3 antibody (modified OKT-3)
Anti-CD25 (Daclizumab, Zenapax) #
NEW
Therapeutic Antibody Center
Roche Pharm. (NIH 02-I-0213)
90% human, 10% murine
Raptiva (Efalizumab, anti-CD11a)
NEW
Genentech and XOMA (In Ph. III
for R.A. etc.)
Amevive (Alefacept, targets selected T-cells) NEW
Biogen For Chronic Plaque
Antegrin (Natalizumab, Alpha-4 Integrin Inhibitor) NEW
Elan Corporation, plc and Biogen,
Inc. (humanized monoclonal AB)
Psoriasis
# It is known by several other names including HAT (Humanized Anti-Tac), SMART anti-Tac, and humanized
anti-IL2-receptor.
See also http://www.bitsplace.com/bitsplace/Autoimmunediscomp.html
Page 14 of 23
(23 - NEWER MONOCLONAL ANTIBODIES UNDER DEVELOPMENT, Cont’d.)
[A recent 2002 study suggests that continuation of anti-TNF-alpha therapy should be
seriously considered if a biopsy confirms the diagnosis of Leukocytoclastic Vasculitis (LCV)
in patients who develop skin lesions suggestive of LCV while being treated with anti-TNFalpha therapy. Other causes of LCV such as infection should be excluded, and the patient
should be evaluated for possible systemic involvement, based on presenting clinical signs
and symptoms. Therapy with glucocorticoids may be useful in causing resolution of LCV.]
24 - NEWER DRUGS UNDER DEVELOPMENT: There is a considerable amount of
research being done for WG, and for various autoimmune diseases. In the near future,
there will perhaps be new drugs to attack specific failures in the immune system regulatory
functions, and also perhaps new tests to better measure disease activity.
LF 15-0195 is a new immunosuppressive by Laboratories Fournier in a small clinical trial for
arthritis and systemic vasculitis. No results to date.
A 1999 study reported successful use of deoxyspergualin (DSG) in three cases of WG. Two
patients achieved remission and maintained remission for the study periods (1 year and six
months respectively). The third patient achieved partial remission during the study period (3
months to date of publication).
In 1998, Prof. Loic Guillevin from Paris elegantly summarized the multi-centered French and
now multi-national studies looking at different treatment modalities in the vasculitides. These
included oral versus intravenous Cyclophosphamide, with or without aphoresis, together
with anti-viral agents such as Vidarabine or Interferon. More recently studies have been
done with Lamivudine. Long-term outcome of his studies is awaited with interest.
Other Possible Advances:
 A stable soluble inhibitor of IgG-FcgR binding offers the possibility of a new strategy
in the treatment of ANCA associated vasculitis syndrome.
 A recent study suggests that ICAM-1 and L-selectin might be therapeutic targets as
anti-adhesion therapy for vasculitis.
 Recent preliminary studies of anti-Migration Inhibitory Factor (MIF) indicate possible
application to autoimmune conditions as well as perhaps a measure of disease
activity.
 Pentoxifylline, a blood thinner, may improve the flow of blood through smaller
vessels. It is used to reduce leg pain caused by poor blood circulation.
 AGIX-4207 by AtheroGenics, Inc. is orally administered, selective modulator of
TNF-alpha, that blocks a subset of TNF-alpha induced activity.
 CCR5 seems key to inflammatory processes and may represent a target for
therapeutic intervention.

The p110delta signaling pathway holds potential for controlling B and T cell
proliferation.
Page 15 of 23
25 - RESCUE THERAPIES: For immediate life threatening cases, plasmapheresis and/or
Immunoglobulin are used in at least some cases. Plasmapheresis essentially replaces the
patient's blood plasma, thus removing the harmful antibodies.
Also, stem cell transplant (SCT) has been used in at least two WG cases and may be a
good choice of treatment for selected cases, although it has significant risks. Another risky
treatment that hasn't been used for WG but has for other autoimmune diseases is immune
system ablation. That can be followed by stem cell transplant, or can depend on bone
marrow to rebuild the immune system. B cell depletion is an alternative to immune system
ablation.
Various immunoadsorption devices such as the recently approved "Prosorba column" or
similar (such as Excorim) may be effective in removing harmful antibodies. In February of
2001, some success was reported using Excorim or Therasorb in early stages of the
disease.
A study compared plasmapheresis with immunoadsorption (Prosorba column?) onto
staphylococcal protein A in patients with ANCA positive vasculitis. In the in vivo study,
immunoadsorption effectively eliminated ANCAs regardless of the ANCA IgG subclass
distribution. Clinically, there was no difference between the plasmapheresis and
immunoadsorption group although the immunoadsorption group had higher initial ANCA
titters.
For information on the immunoabsorption devices available in Europe, scroll down to
Table 1 at http://www.uninet.edu/cin2000/converences/braun/braun.htm
Hyperbaric oxygen may have been used to treat claudication (poor blood flow, particularly in
extremities) leading to peripheral neuropathy.
26 - OSTEOPOROSIS & PERIPHERAL NEUROPATHY: Because a corticosteroid is
almost always prescribed, patients are usually put on Fosamax [Alendronate, or equivalents
Didronel (Etidronate), Aredia (Palmidronate), Clondronate, Ibandronate] or Miacalcin nasal
spray, and also calcium supplements and extra vitamin D to prevent bone loss, and
frequently vitamin and mineral supplements as well.
The new medication Actonel (Risedronate) is available, and may be available as injectable
for use every three months. A WG patient on Prednisone probably should have a bone
scan done initially and annually to check on possible osteopenia or osteoporosis.
Ibandronate has been studied to date in clinical trials involving more than 9,000 patients.
The ongoing clinical development program is evaluating monthly oral and **quarterly
intravenous dosage** regimens in women with postmenopausal osteoporosis. It may be
approved soon.
Peripheral neuropathy can be merely uncomfortable, or very painful. Sometimes the
condition improves over time. Some find relief using the following singly or in combination:
Neurontin, Zonegran, Pamelor, Amitriptyline.
Page 16 of 23
27 - ULCERATIONS: When ulcerations are slow to heal, use of hyperbaric oxygen may be
helpful. Also an expensive cream, Regranex, may be effective. Another (experimental)
drug that may help is Pletal (used for relief of claudication). Sometimes mouth ulcers are a
side effect of Methotrexate. Added folic acid in the diet or by supplement may help, but ask
your physician.
28 - FOLLOW-UP: Before and after remission, WG patients require life-long follow-up to
make sure there are no "hidden" relapses, especially in kidneys and lungs where WG may
be without symptoms until serious damage has occurred. Blood and urine lab work is
perhaps weekly at the start, but tapers as remission is achieved and sustained. Quarterly or
semi-annual checks of key indicators might be in order after some time in remission in
accord with the physician's judgment.
New or aggravated symptoms should be reported to one's physician(s) right away. New
test procedures may be available in the future to better monitor disease activity.
WG patients with kidney involvement might consider requesting a prescription from their
physician for inexpensive (10 cents per) dipsticks to test urine for protein (albumin-specific)
and blood, and report to their physician any positive results. These tests should be done
frequently, perhaps bi-weekly for WG patients in remission, even those without known
kidney involvement.
The table at http://www.hocks.com/urinetest/stripchart.htm compares various test strips and
indicates for which components the particular strip tests. The only ones that seem to have
blood and protein, but not lots of other factors are:
Roche Chemstrip 4 OB
$24.00 per 100 (Blood, protein, leukocytes, glucose)
Bayer Hema-Combistix
$59.99 per 100 (Blood, protein, glucose, pH)
Bayer Uristix 4
$24.00 per 100 (Blood, protein, leukocytes, nitrate)
LW-URS5 (Mfg. Unknown) $13.99 per 100
(Blood, protein, glucose, pH, ketones)
All test for blood and protein. Costs may vary from source to source. Prices shown are for
an on-line pharmacy in October 2002. Shipping costs may be extra.
For lung involvement a CT scan or X-ray annually might be in order. Periodic tests for CBC,
creatinine, BUN, cANCA, anti-PR-3, pANCA, anti-MPO, ESR and CRP are usual to monitor
disease activity and effects of medications. An annual bone scan may be appropriate.
Page 17 of 23
29 - SUBGLOTTAL STENOSIS: The following seem to be the ways that subglottal
stenosis (narrowing of the trachea) has been treated:
TABLE 6
SOME METHODS OF TREATING SUBGLOTTAL STENOSIS
Method
Comment
DILATION
Often requires repeated dilations
DILATION followed by local injected or
Often requires repeated treatments
topical steroid
DILATION followed with topical antibiotic Under study
(Minomycin?)
DILATION with SILASTIC STENT TUBE
LASER SURGERY
Perhaps being used less than in the past
TRACHEOTOMY & Placement
Perhaps subject to infection
of Trach Tube
SURGICAL RECONSTRUCTION
Major surgery
(29 - SUBGLOTTAL STENOSIS, Cont’d.)
The use of laser surgery may be decreasing, as some physicians seem to think it leads to
more scar tissue, and further narrowing requiring still further dilations and/or surgeries.
(Other than WG, there are three other causes of sub-glottal stenosis:
relapsing polychondritis, and amyloidosis).
rhinoscleroma,
After placement of a trach tube, some patients have difficulty speaking or swallowing. The
Passy-Muir Speaking Valve fits on the end of the tube or the vent. It is a one-way valve that
allows effortless speech because the exhaled air can only be directed through the upper
airway. It can also help with pharyngeal pressures during swallowing.
30 - NASAL IRRIGATION: Many WG patients have trouble with nasal crusting, during
healing, and even in remission. Nasal irrigation once or more a day with an appropriate
sterile solution seems to work well for some, to prevent crusting. It's possible nasal
irrigation may help prevent relapses (though there's no studies to show that).
One can consider getting a WaterPik and a "Grossan Tip". The "Pik" should be adjusted to
the lowest flow setting. Use a sterile solution without preservatives. You can mix your own.
Or use BreathEase. See the info at: http://www.pharmacy-solutions.com/ and at
http://www.sinus-relief.com/sinusrelieffaq.html
Two newer devices for nasal irrigation have appeared recently. No feature/cost comparison
is readily available to assist in deciding which to use.
The Interplak oral irrigator. (http://www.interplak.com/) can use the Grossan tip.
The HydroPulse nasal irrigator (http://www.pharmacy-solutions.com/hydropulse.pdf)
There is a nasal emollient called Ponaris that thins the nasal secretions. It is made by
Jamol Laboratories, Inc. in Emerson, New Jersey. A pharmacy can special order it for
about $12.00 for a months supply. This may prove helpful (but only if one's physician
approves the use).
Page 18 of 23
(30 - NASAL IRRIGATION, Cont’d.)
Some WG patients find "Bactroban" used nasally helps control infections. The European
Vasculitis Study Group (EUVAS) has an on-going trial (MUPIBAC) to determine the
effectiveness of eliminating Staphylococcus aureus from nasal passages using Mupirocin
nasal ointment.
Methicillin-resistant-Staphylococcus-aureus (MRSA) is a common infectious organism,
readily infecting persons in hospitals and nursing homes. It is not controllable by most
antibiotics. It may require Vancomycin or a combination of antibiotics to rid the patient of
the infection. It can be guarded against by common precautions, especially not touching
one’s nose, eyes, face, or mouth, and by frequent and thorough hand washing or use of a
topical sterilizing fluid.
31 - DIAGNOSTIC TESTS: WG is tentatively diagnosed by symptoms and by a blood test
known as the cANCA test (90+% accurate for serious cases, 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 is
usually confirmed by biopsy. A related test, the pANCA test, if positive may an indicator for
some other types of autoimmune diseases e.g. microscopic polyarteritis (MPA). The
cANCA and pANCA are immunofluorscent (IIF) tests requiring a human interpretation.
The correlation between cANCA and WG activity is only about 30%, so it's not dependable
in tracking WG activity after diagnosis, though a rise in cANCA/anti-PR-3 sometimes
precedes a relapse.
A newer (ELISA) test, the anti-Proteinase-3 antibody test (anti-PR-3) is somewhat more
specific to WG and perhaps more sensitive also. The anti-PR-3 test may be somewhat
more reliable in determining disease activity than is the cANCA test. It is possible but
unusual to have active disease and be negative in one or both tests (cANCA and
anti-PR-3). Another ELISA test is anti-MPO which can be indicative of several autoimmune
diseases.
At least one recent study shows that in diagnosis both cANCA by immunofluorescence and
anti-PR-3 by ELISA result in greater sensitivity than either test alone. Warning - To get an
accurate result you may have to be off corticosteroids and/or immunosuppressives
for some time. Ask your physician about that.
Different labs may give differing results on the key WG tests, cANCA and anti-PR-3, so it is
best that all these tests be done at the same lab. In the case of ANCAs, it is important to
know at what level of dilution the result is considered negative. A survey done a few years
ago of labs doing the cANCA test showed widely differing results from the same sample
supplied to all the surveyed 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.
More recent work shows that there are varying epitopes of ANCA, which are not all detected
easily by the usual test methods. The so-called “Capture ELISA Anti-PR-3 Test” can be
more sensitive in detecting the less usual epitopes. In particular, the Capture ELISA using
the MoAb 12.8 antibody was found to have a 96% sensitivity in one study,
Page 19 of 23
(31 - DIAGNOSTIC TESTS, Cont’d.)
The new PolyTiter Immunofluorescent Titration System designed to aid in the diagnosis and
management of autoimmune diseases may prove to be better than cANCA but that remains
to be proven, especially in light of the development of the Capture ELISA.
Biopsy is often required for a firm diagnosis but there seems to be a growing tendency to
treat for WG if tests, symptoms, and examination indicate it is likely. Biopsies can be
negative due to the difficulty in sampling the exact tissue, even while the disease is active.
While the standard at NIH seems to be a positive biopsy, individual practitioners may begin
treatment for WG without a positive biopsy.
In patients with both new and past airway involvement, fibrobronchoschopy (endoscopic
exam) for evaluation of the entire airway should be performed.
A recent article in "Lupus" suggests testing for anti-cardiolipin antibodies when ANCA test
return a positive result. Anti-phospholipid antibodies might also be checked.
One recent study indicates quantitative measurement of CD64 can distinguish between
systemic infection and the flare of autoimmune diseases.
There apparently is correlation between WG activity and the levels of some cytokines,
sICAM-1 and Interleukin-6. These could conceivably lead to tests for more accurate
monitoring of WG activity. Also, the CCR5 cytokine levels correlate with disease activity
and may in the future provide another way to track the disease.
Procalcitonin (PCT) level seems to be a useful marker both for the differential diagnosis
between bacterial infection and inflammation, and for the detection of superimposed
bacterial infection in patients with systemic inflammatory diseases. Commercial test kits
may not be readily available for testing PCT levels, and the use of procalcitonin may not be
scientifically sound.
ANCA test results can be positive due to conditions other than autoimmune diseases.
Amebiasis, subacute and bacterial endocarditis have been shown to cause transient
positive ANCAs.
Because WG can have clinically silent cardiac involvement, echocardiography might be
appropriate at times, and certainly should be considered if there is any sign of cardiac
abnormality or dysfunction.
An unusual number of endothelial cells circulating in peripheral blood can be a marker for
small-vessel vasculitis. Anti-Endothelial Cell Antibodies (AECA) are involved in some
vasculitides and may be useful indicators of disease activity.
An M/S Word file on ANCA testing methods can be downloaded from:
http://mail.chartermi.net/~blader/ANCA-Detection.doc
A file of information from the AARDA on the diagnosis of immune diseases may be
downloaded from: http://www.chartermi.net/~blader/Diagnosis-AARDA.doc
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32 - SIMILAR DISEASES: WG is one of a number of vasculitis diseases. Vasculitis is
caused by infection, medication reactions, "connective tissue disorders", or other unknown
causes.
Because of overlapping symptoms, deciding on the exact autoimmune disease is only partly
science. Different physicians may reach different conclusions, which of course confuses
patients no end.
The treatments for various autoimmune vasculitides are somewhat similar. Depending on
the patient's condition, some physicians at times deem it prudent to start treatment before
an absolutely firm diagnosis has been made, especially in cases that are life or organ
threatening.
Others that may have some symptoms similar to WG are:
TABLE 7
PREDOMINANTLY AFFECTING LARGE- and MEDIUM-SIZED BLOOD VESSELS
Giant Cell Arteritis (GCA)
Takayasu’s Arteritis
Temporal Arteritis
PREDOMINANTLY AFFECTING MEDIUM-SIZED BLOOD VESSELS
Polyarteritis Nodosa (PAN)
Kawasaki’s Disease
PREDOMINANTLY AFFECTING MEDIUM- and SMALL-SIZED BLOOD VESSELS
ANCA POSITIVE
Churg-Straus Syndrome
(CSS)
Wegener’s Granulomatosis
(WG)
# Microscopic Polyangiitis
# Sometimes identified as microscopic polyarteritis
PREDOMINANTLY AFFECTING SMALL-SIZED BLOOD VESSELS
ANCA NEGATIVE
Henoch-Schoenlein Purpura (HSP)
Essential cryoglobulinemic vasculitis
Leukocytoclastic Vasculitis
Anti GBM disease
(Goodpasture's disease) ##
## Some may have positive ANCA
DISEASES THAT MAY BE AUTOIMMUNE VASCULITIDES
OR HAVE SOME SYMPTOMS SIMILAR TO WG
Hypersensitivity Vasculitis
Rheumatoid Vasculitis
Relapsing Polychondritis
Buerger's Disease
Cogan's Syndrome
Central Nervous System
Vasculitis (CNS)
Necrotizing Sarcoid
Granuloma (NSG)
Polymyalgia Rheumatica
Behcet's Disease
OTHER AUTOIMMUNE DISEASES THAT MAY HAVE SOME SYMPTOMS
SIMILAR TO WG, OR ARE PERHAPS SUPERSEDED TERMINOLOGY
Necrotizing Respiratory Granulomatosis
Lupus Erythematosis
Pathergic Granulomatosis
Lethal Midline Granuloma
Page 21 of 23
(32 - SIMILAR DISEASES, Cont’d.)
Present classification of autoimmune vasculitides does not seem to be comprehensive and needs
further clarification.
An M/S file is available on additional information on classifications and clinical symptoms of
autoimmune vasculitides is available from:
http://pws.chartermi.net/~blader/ Vasculitis-Classifications.doc
33 - NATIONAL INSTITUTES OF HEALTH: The National Institute for Allergy and Immune
Diseases in Bethesda, Maryland has long running studies on WG. To be admitted, a patient
must be referred by one's attending physician, have active WG, and a positive biopsy. After
referral, the first trip to Bethesda is at the patient's expense.
Further information on NIH clinical trials can be obtained from:
http://clinicalstudies.info.nih.gov/
For contact information in the Division of Allergy, Immunology, and Transplantation (DAIT)
http://www.niaid.nih.gov/cgi-shl/contacts/contacts2.crm?abbreviation=DAIT
or
http://www.niaid.nih.gov/dir/labs/lir/sneller.htm
Once accepted into the study, some expenses are paid by the NIH including medications,
travel etc. The NIH continues to work with the patient's local physician(s).
An M/S Word file on searching the National Library of Medicine Medline database and other
Internet resources (not links) may be downloaded from:
http://pws.chartermi.net/~blader/Medline-Search.doc
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34 – REFERENCE TO CYTOXAN AND PREDNISONE SIDE EFFECTS:
file on side effects of Prednisone and Cytoxan can be downloaded from:
An M/S Word
http://mail.chartermi.net/~blader/ - 34 - SIDE EFFECTS
Note - Most patients experience far fewer than the lists for both drugs.
It is very difficult for patients and physicians to tell what particular symptoms are caused by
the disease, and those caused by medication side effects. One should keep one's
physician aware of changes in current symptoms, as well as new ones.
35 – REFERENCE WEB PAGES (URLs):
A file of WG, related medical, & SSI/SSDI web addresses is available at:
http://mail.chartermi.net/~blader/WG-URLS-V1.html
36 - MEDICAL TESTS AND WEGENER'S GRANULOMATOSIS
An M/S Word file of information on medical tests used in diagnosing and treating WG Is
available at:
http://mail.chartermi.net/~blader/Wegener's-Tests.htm
- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -- - - - - - - - - - - - - - Please forward comments and corrections to blader@chartermi.net
Web Page: http://mail.chartermi.net/~blader
(Page created by a Wegener's Granulomatosis patient)
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