Dr. Kardas.. I'm sorry you or someone else has been diagnosed with
Wegener's granulomatosis. I'm posting this to the newsgroup, and
also copying you directly to make sure you get the reply as quickly
as possible.
I'll answer your questions first:
1. For further information, go to http://www.wgsg.org and read the file
I'm attaching.
2. Early diagnosis and treatment are critical to preventing organ
damage.
3. Serological tests are the cANCA (and related anti-PR-e), and
sometimes pANCA (and related anti-MPO). The anti-PR-3 test is considered
somewhat more selective and sensitive than the cANCA alone. The exact
criteria for classifying as "positive" may vary from lab to lab. I
currently have a cANCA 1:32 which is considered low, but positive. The
anti-PR-3 from the lab I use is 0.0-0.9 negative, 0.9-1.0 ambiguous, 1.1
and above, positive. Mine is currently 2.25 and I have a minor flare.
4. The 'gold standard' for diagnosing WG is airway involvement, a
positive cANCA (out-dated?), and a positive biopsy. However, the
consequences of not treating WG are serious enough so that many or
perhpas even most physicians will start treatment when other obivous
alternative diagnoses have been eliminated, and when the patient symptoms
seem strongly indicative of WG.
5. I can't recommend hospitals in Germany, only in the U.S. According
to the latest U.S. News and World Report ranking of hospitals for
rheumatology, Mayo Clinic, Johns Hopkins and some other well known
clinic/hospitals are ranked tops. It is strongly recommended that you
see a physician with substantial experience in diagnosing and treating WG
as it is as much an art as a science.
I'm attaching a long file on Wegener's granulomatosis. Please consider
joining the WG Support Group in Kansas City, Missouri for there patient
packet and bi-monthly newsletter. Also, consider subscribing (free) to
the e-mail list at
http://www.weareb.org/WG/howto.html#usage
Note there is a live chat on Internet Relay Chat on Monday evenings
starting at 9 PM EDT. It is on Newnet. The channel is #wegener (no 's',
no apostrophe). Instructions at the web address above.
Good luck on your diagnosis and treatment. If caught early enough, the
treatment isn't too severe, but is lengthy. As you probably know, the
disease is quickly fatal (months) if not treated.
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INFORMATION ON UNUSUAL DISEASE July 30,1999
Persons with PERSISTENT sinus/nose/ear/throat problems
which do NOT respond to NORMAL treatments might consider
the possibility of an autoimmune disease by seeing a
rheumatologist broadly experienced in autoimmune diseases.
There is an autoimmune disease, Wegener's Granulomatosis
(WG), 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 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.
About 1 in 40,000 develop the disease. It can strike at any
age, and equally among both sexes (unlike many autoimmune
disease which have a higher percentage of female patients).
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.
PERCENT OF PATIENTS WITH SYMPTOMS
Symptom Incidence At Total (%)
Onset (%)
--------------------------------------------
Ear, Nose, Throat 75 95
Lung 50 85
Joints 30 70
Fever 25 50
Kidney 20 75
Cough 20 50
Eye 15 50
Skin 15 45
Weight Loss 10 35
Pericarditis 5 10
Peripheral Nerves 0 15
Central Nervous System 0 10
WG affects one in every 30,000 to 50,000 people.
WG can occur at any age.
It 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, and 1% are of another race.
WG affects males and females equally.
SYMPTOMS AFTER REMISSION
Symptom Patients (%)
Chronic renal disease 40
Hearing loss 35
Cosmetic nasal deformity 30
Hoarseness and tracheal
stenosis 15
Visual loss 8
WG is not contagious nor hereditary in so far as is known,
though a tendency to autoimmune diseases seems to run in
some families.
WG is fatal unless treated. With treatment, most patients
can lead a normal or nearly normal life.
Most physicians have never diagnosed a case of WG. It is
best to find a rheumatologist or internist who has exper-
ience with the disease. The average time from onset of
symptoms to diagnosis is 5 months, though some suffer years,
with severe damage to organs before being correctly
diagnosed. Many WG patients have had numerous visits to
ENT specialists and other specialists before being correctly
diagnosed.
Although the disease remains basically incurable, there are
several effective treatments. 95% of WG patients are able
to achieve remission which may last years or months.
"Standard" treatment originally was Cytoxan and Prednisone
for about one year (the "Fauci" regimen). More recently,
some use Cytoxan and Prednisone until the disease starts to
abate, then substitue methotrexate for the Cytoxan until
remission. The prednisone dosage is frequently tapered
very carefully as quickly as possible after the initial
acute phase.
In Germany there is apparent use of intramuscular depot
steriod every three weeks as opposed to daily oral use.
A new drug Deflazacort (Azacortid and Calcort) is under
study but not yet approved by the FDA. It may replace
prednisone in some cases as it appears to have less side
effects. It perhaps is being used in Canada, Mexico and
Europe.
Light cases of WG without serious organ involvement may use
only Bactrim (Septrim) and Prednisone, though opinions on
this treatment vary. Unusually a patient may go into re-
mission on prednisone alone.
In place of Cytoxan (in some countries), other treatments
substitute Cellcept (Mycophenolate Mofetil), Sandimmune
(Cyclosporin), Imuran (Azathioprine), or Arava (Leflunomide).
For immediate life threatening cases, immunoglobulin
and/or plasmapheresis are used in at least some cases.
I believe that some physicians in some countries may be
using Leukeran (Chlorambucil) in treatment of WG. Another
drug used sometimes is Campath. Also used is Muromonab-CD3
(also known as OKT3), which may not be legal for use in the
U.S. Both the above are monoclonal antibodies, one of a
family of drugs under development that promise to be effective,
but perhaps expensive.
Newer drugs IL-10 and FX506 are in various stages of investi-
gation, but are not yet be FDA approved to my knowledge
Because prednisone is almost always used, patients are
usually put on Fosamax (alendronate, or equivalents
clondronate, ibandronate and etidronate) and calcium
supplements and extra vitamin D to prevent bone loss,
and frequently vitamin and mineral supplements as well.
WG is tentatively diagnosed by a blood test (95% accurate)
known as the cANCA test. Not all labs do this test, Mayo
Clinic lab being one that does. A tentative diagnosis is
usually confirmed by biopsy. A related test, the pANCA test
if positive may an indicator for some other types of auto-
immune diseases e.g. microscopic polyarteritis (MPO).
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, and 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.
The newer test, the anti-Proteinase-3 antibody test (PR-3) is
somewhat more specific to WG and perhaps more sensitive also.
Not all medical labs do the test so it is a 'sendout' test at
most labs. The 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).
The cause of WG is not known. The immune system produces too
much or the wrong type of antigens which then cause neutrophils
(white blood cells) to attack the endothelial cells which form
the lining of blood vessels.
Bacterial and/or viral infection appear to be involved in the
development of the disease but proof is still lacking. There
are indications that a sinus infected with Golden Staph seems
to trigger relapses in some WG patients.
There are other autoimmune diseases some of which have some
symptoms similar to WG Some of these are:
Lupus Eurythematosis
Lethal Midline Granuloma
Necrotizing Respiratory Granculomatosis
Pathergic Granulomatosis
WG is one of a number of vasculitis diseases. Vasculitis
is caused by infection or "connective tissue disorders"
Others which may have some symptoms similar to WG are:
Giant Cell Arteritis Polymyalgia Rheumatica
Beh?et's Disease Takayasu's Arteritis
Cryoglobulinemia Churg-Strauss Syndrome
Hypersensitivity Vasculitis Rheumatoid Vasculitis
Relapsing Polychondritis Henoch-Sch?nlein Purpura
Cogan's Syndrome Microscopic Polyangiitis
Buerger's Disease Kawasaki's Disease
Leukocytoclastic Vasculitis Central Nervous System Vasculitis
Polyarteritis Nodosa
There is an excellent WG support group which puts out a bimonthly
newsletter for an annual due of $15 ($20 outside U.S.). (Tax
deductable donations gratefully received).
Wegener's Granulomatosis Support Group (WGSG)
P. O. Box 28660
Kansas City, Missouri
64188-8660
1-800-277-9474
1-816-848-4444 (fax/phone)
E-mail wgsg at wgsg.org
The WGSG also have available on request a packet of information
for patients, and also on request, a physicians packet of
information.
Their web page at http://www.wgsg.org has lots of useful in-
formation and links. Please e-mail the WGSG at wgsg at wgsg.org
if you have questions or concerns about this disease. An
e-mail group (listserve group) has about 250 subscribers.
Information is at http://www.weareb.org/WG
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 (Mac) to connect.
There is a WG Message Board at
http://www.InsideTheWeb.com/messageboard/mbs.cgi?acct=mb21847
Another WG message board at
http://www.support-group.com/cgi-bin/sg/get_links?wegeners
seems to get little use.
The National Institute of Health in Bethesda, Maryland has a
long running a study 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.
Once accepted into the study, most expenses are paid by the NIH
including medications etc. The NIH continues to work with the
local physician.
P.S. I'm not a physician. What I've written above has been
derived from experience and various sources. I can't be held
responsible for the accuracy of said information. What I have
said is what I've learned in researching my own case, and not
necessarily accurate nor applicable to others.
A Wegener's Patient
