INTRODUCTION TO AUTOIMMUNE VASCULITIS

1 - SOURCES OF THIS WEB PAGE

• The following information is derived from a variety of sources over some eight+ 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 has no medical training. 

• Much comes from Medline abstracts and medical journal articles.  Some is from AV and WG patients and carers, some from newsgroups,  internet web pages, etc. The compiler has attampted to use only valid medical information, but cannot guarentee the validity in every case.
  

• The writer/editor/compiler does not vouch for the accuracy, completeness, nor applicability of the information below to any person, whether an AV patient or otherwise.  No medical decisions should be made on the basis of information on this web page or on associated linked documents and web pages unless those are from a recognized medical professional or publication.

•  ALL MEDICAL QUESTIONS, SYMPTOMS, CONCERNS AND PROBLEMS SHOULD BE DIRECTED TO APPROPRIATE LICENSED MEDICAL PROFESSIONALS. 
  
• To view a list of 76 questions relating to finding a physician, diagnosis, and treatment, see Section 7 - THINGS TO ASK ABOUT VASCULITIS

3 - FEELING ALL ALONE? 

• Contact the Vasculitis Foundation [VF] in Kansas City, Missouri, USA
  

• The VF is donor and member supported.  Please help keep this invaluable organization funded by donations, bequests, and memorial gifts. 
  
• Membership will get you the bi-monthly VF newsletter.  The annual dues are $US 25 (domestic) or $US 30 (foreign).
  
• Each newsletter carries a list of contact persons for various locations.
  
• You can join by phone, e-mail, or on the VF web page.  See above address information.
• Persons unable to afford the annual dues can request the newsletter to be supplied at no cost.
• Donations can be made on line at the VF web page at http://www.vasculitisfoundation.org/donate
  
• Bequests are welcome and needed.  Types of bequest are various.
• You can give almost any kind of asset to the Vasculitis Foundation through a bequest, including cash, securities, an interest in real estate (such as a residence), tangible personal property (such as works of art or antiques) or the remainder of your IRA, Keogh, tax-sheltered annuity, qualified pension or profit-sharing plan.
• Ask your attorney about your bequest options, such as specific bequests, residuary bequests, testamentary trusts and QTip trusts.
  

• Register with the Vasculitis Clinical Research Consortium (VCRC) at http://www.rarediseasesnetwork.org/vcrc/index.htm
• The VCRC can enroll vasculitis patients in clinical trials.  The advantages of a clinical trial are:
• The patient may receive the latest medications as part of the care
  
• The patient will receive medications at no cost
  
• The patient are followed very carefully by physicians expert in their specialties.
• Enrolling in a clinical study will further the understanding of WG and various treatments.
  

• Ask the VF for references to physicians and hospitals that specialize in vasculitides.
• Currently the VF doesn't maintain a list of physicians, hospitals, and clinics that specialize in AVs, but maintains a list of VF consultants.
• For now, patients have to utilize the experiences of other patients to find an AV expert in a particular area.
  
• Also ask the VF how to contact a patient's support group for the particular vasculitis you have.
• Support group members can be help find expert physicians near you in many cases.
  

• The VF conducts a bi-annual Symposium every even-numbered year.
• The VF Symposium is an excellent way to meet many AV and WG patients.
• Patients and advocates can learn from the many presentations by leading researchers and clinicians.  
• The 7th International WGA & Vasculitis Symposium was in Baltimore, Maryland July 7-9, 2006. 
  
• For details, see March/April or later issues of the VF newsletter.
  
• The 6th International Wegener's Symposium was at the InterContinental Hotel adjacent the Cleveland Clinic, in Cleveland, Ohio August 6-8, 2004.  
  
• For information on that and past symposia, refer to past editions of the VF newsletter and to the VF web page at http://www.vasculitisfoundation.org 
• Videos and DVDs of past symposia are available from the VF.
• The 8th International Vasculitis & WG symposium will be in Rochester, Minnesota in Summer, 2008.
  

• A number of email groups provide for support and information to vasculitis patients.
• Click here to view guidelines for using email groups and newsgroups.
• Many can be found by searching Yahoo and MSN groups.
• There may be many other listserv and majordomo supported groups that don't appear on searches.
• There may be many other listserv and majordomo supported groups that don't appear on searches. 
  
• To join or access email groups on Yahoo, you have to have a Yahoo identity and password.
  
• To find email group pages,go to http://health.dir.groups.yahoo.com/dir/Health___Wellness
• Search for the particular group subject. 
  
• When the search results come up, click on the name of the one you want. 
  
• When the group page comes up, click on “Join This Group”
• Some of the many groups for all autoimmune vasculitides are:
• Rheumatoid_Vasculitis
• savvy  (has many members with CSS)
• vasculitis-discussions
• VasculitisSurvivors
• vasculitisupport
• Vasculitissupportgroup

• Some of the many groups for specific AVs are:
• Central nervous system vasculitis (CNSV)
• Churg-Strauss syndrome (CSS)
• Giant cell arteritis (GCA)
• MPAvasculitis (MPA)
• Polyarteritisnodosa (PAN)
• Takayasu's arteritis (TA) (Takayasu’s Disease)
• Takayasu's arteritis (TA) (Takayasu’s arteritis)
• Wegener's Granulomatosis
• wgidiscussion
• wegeners4parents
• wegeners-teens-only
• wegeners4weightloss
  
• Wegeners_Granulomatosis (VCRC sponsored)
• Wegeners_Disease (European's only)
• Wegener's Granulomatosis (WG) 

• Click here for a slightly more complete list of email groups concerning autoimmune vasculitides.

• Click here to view an autoimmune vasculitis reading list for patients and others.

• Three public "MySpace" groups are:
• "Wegener's" with 32 member (on 25 July '07)  at http://groups.myspace.com/index.cfm?fuseaction=groups.groupProfile&groupID=100910512
• "Wegener Granulomatosis" with 53 members as (on 25 July '07) at http://groups.myspace.com/index.cfm?fuseaction=groups.groupProfile&groupID=105189328
• Also a new "MySpace" group, "Weggies4life" with 4 members (on 25 July '07) at http://groups.myspace.com/weggies4life
• The memberships shown may not all be WG patients or carers.  If you post to these, be sure to remain anonymous (no last names, addresses, emails, etc.)
  

• In the presence of an advocate, your physician may spend more time discussing your case more thoroughly and allow more questions.
  

•  When one is ill, he or she is hardly in a position to ask the right questions, nor to understand nor remember the answers. 
• If possible, take an advocate with you to every appointment to take notes, ask questions, write down answers, and remember the details that later you will have already forgotten.
• If without accompaniment, your physician might permit recording appointments
  
• Your advocate should help you prepare a list of questions before each appointment.
  
• Before leaving the office, ask for a copy of the medical  report be mailed to you.
  

• Investigate whether your health insurance company will provide you with a patient advocate when dealing with hospitals.
• Some hospitals have  patient advocates on staff.  In some cases a hospital social worker may be of assistance.

• An advocate needs to be aware of what the patient is experiencing both physically and emotionally.
• An advocate can be helpful in buffering the  vasculitis patient from unhelpful family and friends, and perhaps help mend strained relationships.
• Pain is often difficult for patients and for advocates.
  
• Click here to view a file with information on dealing with persons in pain.
• Many AV patients have clinical depression at times.
• Anti-depressants may be suitable and effective.
• If one type isn't, then have your physician prescribe different ones and experiment with dosages until you find one that works.
• Students in school need an advocate.
• The medications used to treat AVs cause many symptoms that interfere with learning. 
  
• The students' parents need to work with school personnel to get a mutual understanding of the difficulties a student faces and the need for special treatment, a written plan that the school and parents agree to.
• Click here to download a Word trifold brochure on "Chemotherapy And  Education", prepared by parents, and students and adults with an autoimmune vasculitis.
• Parents and  teachers need to be advocates for the child being treated with chemotherapy for cancer or vasculitis.

5 - RECORDS YOU NEED

• Click here to see what records you must carry on your person at all times.
  

• Before you get involved in the process of diagnosing and treating any serious disease, it is important to buy a notebook and keep a daily journal (or blog if you want to be public).
  
• The journal will help you track the disease symptoms and medication side effects that are otherwise easily forgotten. 
  
• You and your advocate should always prepare a list of questions for your next appointment and keep the questions and answers in your journal.

• Write down EVERYTHING!  
  
• It is your responsibility to keep complete and up to date records regarding your health care,  as you'll likely deal with many different physicians, labs, clinics, and hospitials over the years,  .
  
• Date and time should be recorded with every entry.
• Data should include all current and recent symptoms, every office visit, every hospital stay, every test and test result. 
  
• Keep a medication log with start and end dates and dosage changes.  and the effects that they have on you.
  
• Before every lab and radiology test, request the lab mail you the radiologist's report directly. 
  
• In the United States  and some countries you have a legal right to copies of  the records.  
  
• In other countries it may be the physician's option to release  medical records to you. 
• You may be charged for the cost of making copies.
• See the Johns Hopkins Forms web page for forms for a both Medications Form and a Medical Contact form at  http://vasculitis.med.jhu.edu/resources/forms.htm  
  
• If you have one, consider using your computer for record keeping, both text and spreadsheets.
  
•  See information on medical records at http://www.beatcfsandfms.org/html/GeorgesOrganization.html
  
• Always request a copy of the physician's appointment record be mailed to you.

• Radiology films may be destroyed after some years.  In order to have a complete radiograph history, order a copy of the film at the time of the tests.
• Ask about the length of time that computerized images are kept on file.  If a limited time, request a copy of the computerized record, or a film copy.
  

• Click here to learn why you should have copies of all your medical records. 

• The Medical Information Bureau (MIB) is a centralized repository and clearing house for medical reports from insurance companies.
  
• To view information on how to obtain your record from the MIB, click here.
• Insurance companies use the MIB in making decisions about coverages. 
  
• When information is incorrect, follow the MIB procedures to get the information corrected.
  

• Vasculitis is a condition where the body has inflammation in the blood vessels or vessel walls.
  

• Types of inflammation are: 
  
• Hypersensitivity vasculitis which is an allergic process,
• Vasculitis as a result of infection or perhaps trauma.
• Autoimmune vasculitis in which the immune system is not properly regulated, thereby causing cytokines or other proteins that cause blood cells to adhere to blood vessel walls, and subsequent damage. 
  

• Limits to this web page concerning vasculitis.
• The author/compiler/editor of this document has had NO medical training.
• Only autoimmune vasculitides will be considered, not hypersensitivity vasculitis nor vasculitis as a result of infection or trauma.
• Here after "autoimmune vasculitis" will be abbreviated as AV or AVs for the plural
• Some sections and comments apply only to Wegener's granulomatosis.  Others refer to autoimmune vasculitides in general.

• Causes of vasculitides are unknown, but thought to be  infection, genetical predisposition,  environmental.
• CLICK HERE to view an  html file with a 2006 abstract on the various causes of vasculitis (not all autoimmune)..
• Ross River virus was the first microbe linked with ANCA-positive disease. Other antisense regions complementary to PR-3 included sequences from Entamoeba histolytica, and  Staphylococcus aureus, the two pathogens that have been most closely linked to the development of PR-3-ANCA in humans.
• A 2002 study reported four cases of vasculitis due to antigen inhalation.
• British geneticists have found in 2007 that people with fewer than two copies of the gene FCGR3B are more susceptible to autoimmune disorders, including lupus.
• Researchers at Imperial College London said evidence is mounting that, in addition to gene mutations, variation in the number of copies of individual genes can influence susceptibility to common diseases.
• It was previously shown variation in the copy number of FCGR3B is associated with susceptibility to a kidney disease called glomerulonephritis.
• In the new study, Timothy Aitman and colleagues determined variation in the number of FCGR3B copies is associated more broadly with autoimmune disorders, including lupus, microscopic polyangiitis, and Wegener's granulomatosis.
• The small group of individuals who have no copies of FCGR3B have a dramatically elevated risk of autoimmune disease.
  

• 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].
  
• 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.

• As many vasculitides have symptoms similar to non-vasculitides conditions, it's important for the diagnostician to consider differential diagnoses so that a misdiagnosis will not be likely.
• Click here to view a table of differential diagnoses when considering vasculitides.
• Click here to download a Word table with another table of differential diagnoses.

• A January 2007 study implicated the complement system (IgG) in ANCA related vasculitis..

• To understand what is involved in diagnosing and treating autoimmune vasculitides, one needs to ask the right questions of their physician(s).

• Click here to view a file with a list of 76 questions, each with a bit of background on why they might be asked.
• These questions are NOT intended to be presented directly to a physician. 
  
• The sheer number of questions would likely result in a negative response.
  
• They are intended to be used to help the patient or advocate write up 5-10 questions for each upcoming appointment as seem applicable.
• The background comment with each question is to help the patient know why the question is appropriate at times.
  

• For information on all the AVs, see National Library of Medicine MedlinePlus at http://www.nlm.nih.gov/medlineplus/vasculitis.html   
• The following web pages are readily accessible and understandable:
• Vasculitis  (Patient Education Institute) - Interactive tutorial requires Flash Player for audio and visual presentation.
• Also available in: Spanish
• Overview of Vasculitis (UpToDate web site)
• Vasculitis (Mayo Foundation for Medical Education and Research)
• Vasculitis  (Johns Hopkins Vasculitis Center)
• Center for Vasculitis (Cleveland Clinic, Cleveland OH)
  
• Arthritis Research Campaign (U.K.)
• Living with Vasculitis Booklet (University of Birmingham, U.K., requires Adobe Acrobat Reader)    

• Click here to view a page on medical information to be carried on your person at all times.
  

• Even after remission, AV patients require life-long follow-up to make sure there are no "hidden" relapses, especially in kidneys and lungs where AV may be without symptoms until serious damage has already occurred.
  
• Blood and urine lab work is perhaps weekly at the start, but less frequently as remission is achieved and sustained. 
  
• After the disease has been in remission for some time, quarterly or semi-annual checks of key indicators might be in order in accord with the physician's judgment.

• New or aggravated symptoms should be reported to one's physician(s) right away.
  
• Sudden vision changes should probably be considered a medical emergency and so require an ophthmalogist's attention within the hour.
• Giant cell arteritis (GCA) also known as temporal arteritis (TA) can cause vision loss despite blood test results showing CRP and ESR in "normal"  ranges.
  

• New test procedures may be come available in the future to better monitor disease activity.

• Most AV patients (even those without kidney involvement) should purchase inexpensive dip-sticks.
• Several kinds of AVs can affect kidneys.
• These tests should be done frequently, perhaps  bi-weekly for AV patients whose disease is in remission.
  
• Patients with kidney damage may well benefit from use of dipsticks even if they normally have blood or protein in their urine, as increases may be detected.
• Test urine for protein (albumin-specific) and blood.
  
• Getting a prescription from their physician for these might permit health insurance reimbursement.
• If the pharmacy doesn't have the desired type, they may special order them through a wholesale distributor.
• Report to your physician any change in results from previously reported test results.
• Dipstick sensitivity degrades over time.  Ask your pharmacist and physician about when to discard unused dipsticks.
  

• For VF information on dipsticks, search Google or other search engines to find the type desired.
  
• Features of various dipsticks vary in the number of tests with the more tests being the more expensive. 
  
• It's a good idea to test for glucose as well as blood and protein as prednisone can cause diabetes.
  
• Some that seem to have blood and protein, but don't test for many other factors are:
  
  Roche  Chemstrip 4 OB    $24.00 per 100   (From wholesale house, $85 in 2006 (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 #                         $13.99 per 100    (Blood, protein, glucose, pH, Ketones)     # Manufacturer unknown

• All the above test for blood and protein and glucose
  
• Costs may vary from source to source.  Prices shown are for an on-line pharmacy in October, 2002.
• Shipping probably not included.
• This list has not been updated since Oct. 2002, except as indicaed.
•  Features of various dipsticks vary in the number of tests.  Those with the more tests are more expensive.

• Other monitoring tests are needed to detect asymptomatic disease relapse.
• A more exacting test for blood in urine is a laboratory test that centrifuges the urine to separate out the cells and casts.
• For persons with lung involvement or undergoing a relapse, a CT scan, X-ray, or MRI imaging annually are reasonable.
  
• 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 if the patient is on a glucocorticoid such as prednisone or similar.
  

• Persons with one autoimmune disease are predisposed to develop a second one. 
  
• Autoimmune thyroid disease is the most common autoimmune disease in the U.S. (and perhaps other countries). 
  
• A constant feeling of being cold and of lethargy may indicate hypothyroidism.  If one experiences those symptoms, it's best to have one's thyroid function evaluated.
• Recent information indicates that patients do better with restoral of both TH3 and TH4, rather than only TH4.
• New symptoms could conceivably be the result of some autoimmune condition other than a vasculitic one.
  

FIGURE 1- VESSEL SIZES AND TYPICAL AV INVOLVMENTS




FIGURE 1A - PRESENTATION OF VASCULITIDES VERSUS VESSEL SIZES

• Click here to download an introductory slide presentation on Wegener's granulomatosis.

• Brochures describing WG are available on request from the VF in English, Swedish, German, and Spanish. 
  
• Click here to download an Adobe Acrobat file of the VF brochure.
  
• (Future brochures may include other AVs and more languages.)

• Patients and others can download from the VF a 150 page "Patient Packet" in either Adobe Acrobat or Microsoft Word format with good information on the disease and treatments.
• For those who don't care to download the packet, it can be requested from the VF by phone, mail or e-mail.
  
• Also, one can request a "Physician's Packet" for their physicians who aren't familiar with WG.
• The VF web page has a list of VF contacts in U.S. states  and perhaps for provinces or countries with names, cities, and phone numbers listed so a patient can contact someone close to them who is familiar with WG, and perhaps can advise of local support group meetings.
• Click here for the VF web page.  Scroll down to "About VF". and then "Learn More" then select one of the options.
  

• If you are feeling all alone, there is a VF (WGA) E-mail group has 275 subscribers.  The patient's and care-givers there have lots of information, experience, and most importantly, support. 
  
• You can subscribe (at no cost)  at http://health.groups.yahoo.com/group/wgdiscussion/
• Another email group is Wegeners_Granulomatosis with about 150 members, also a Yahoo group.
  
• There is an on-line chat (IRC) on Monday evenings at 9 PM ED/ST (0200 UTC/GMT), in the "wegener" channel on NewNet.
  
• Use an IRC client program such as mIRC (PC)  to connect.
• Go to the mIRC web page (at http://www.mirc.com/)  to download and install the client progrm.
  
• Note the channel is NOT "wegener's", nor "wegeners", but merely "wegener" (but without the ""). 
• Alternatively, one can use the "Chat" button on the Newnet web page at www.newnet.net   
  
• Those using Newnet chat will need to type /join #wegener in the command line at the bottom of the IRC window.

• For information on the future of a WG (and perhaps AV) patients, see Section 15 below

• Persistent sinus-nose-ear-throat-lung or kidney problems that have not responded to normal treatments may be experiencing early symptoms of Wegener's Granulomatosis (WG). 
  

• WG is an autoimmune vasculitis which can be a relatively slow moving disease taking months or years before becoming acute.

• WG can also be very sudden and severe. 
  
• It can do irreversible damage in a short time, but often it smolders before finally becoming acute.
• Kidney function can be severely damaged in just days if not treated properly.
  

• WG can present many other initial symptoms in addition to the usual upper airway symptoms.
  
• Other symptoms might be excessive fatigue, night sweats, joint pains and other arthritic-like symptoms. 
• One fairly distinctive sign is "saddle-nose deformity", where the bridge of the nose collapses due to cartilage destruction.
  
• After remission, this deformity can often be reconstructed (not "cosmetic surgery", but reconstruction).
  
• NOTE - Saddlenose deformity is not always specific for WG. 
  
• It also can occur in relapsing polychondritis and less commonly in rhinoscleroma, leprosy, Takayasu's areteritis, pyoderma gangrenosum, syphilis, atrophic rhinitis, rheumatoid arthritis, Marshall's syndrome (ectodermal dysplasia),
  
• Septum perforation is another sign of either WG or relapsing polychondrities or excessive use of cocaine.
  
• Click here to see more on symptoms in Section 14, "Description of WG".
• Click here to go to Section 21,  "Medical Tests and Wegener's".

(Click here to return to Index)

13 - AN OVERVIEW OF WG

• WITH VERY RARE EXCEPTIONS, WG IS FATAL UNLESS TREATED.

• With early diagnosis and appropriately aggressive treatment, most patients lead a normal or nearly normal life.  The following data in the paragraphs below may be out of date.
  

• It is believed that more patients suffer from under-treatment than over-treatment

• WG is neither contagious nor hereditary in so far as is known. 
  

• Genetic, environmental, and very likely infectious factors are involved in development of  WG.

• Although the disease remains incurable, there are a variety of 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.

(The above figures may be out of date and may represent under reporting and poorer access to health care for lower income persons)

FIGURE 2 - ORGANS MOST FREQUENTLY AFFECTED BY WG

(Click here to return to Index)

14 -  DESCRIPTION OF WG

• WG is a systemic disease, a form of autoimmune vasculitis which affects mostly smaller blood vessels, down to capillary size but  also occasionally in larger vessels.
  
• Vasculitis refers to inflammation of the blood vessel walls, regardless of cause.
• Autoimmune refers to a malfunctioning immune system that produces antibodies that attack tissues and organs rather than destroying invasive infectious agents or allergens.
  
• Systemic means it can affect almost any organ or tissue is more common in certain organs and tissues.
  
• It can lead to cell death and organ impairment or failure. 
  
• It can strike at any age, and equally among both sexes (unlike some AVs that afflict a higher percentage of female patients). 
  
•  It is usual for granuloma to form but not always.  Granuloma generally are closely associated with blood vessels.
  
• Granuloma occur due to  diseases other than  WG.
• The granuloma in WG are predominantly composed of T-lymphocytes and macrophages that infiltrate blood vessel walls and adjacent tissues.

• 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 less often, heart, pancreas, prostate, liver, testicles, and rarely pituitary, penis, or other organs. 
• Recent studies show that silent heart damage occurs in a substantial percentage of WG cases.
• 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.
• A 2006 test for pro-BNB, a blood serum protein, showed that the level of the protein correlates with the extent of disease.
• WG without kidney involvement is known as "limited" WG.  That does not mean it is less dangerous.
• AVs including WG predisposes one's blood to clotting so can occasionally cause stroke or stroke-like symptoms. 
• Any diminution in central nervous system or vision functions and abnormalities should be reported to one's physician at the first opportunity.
• Click here to view a simple three step test to determine if someone has had a stroke.
  

• Estimates of WG frequency vary from about 0.8 to 15 per 1,000,000 but these figures are likely low. 
  
• It seems likely many cases of WG are never correctly diagnosed so 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 reported incorrectly reported as kidney failure etc., rather than vasculitis.
  
• The preliminary results of a 2003 Swedish study showed the frequency of WG to be 156.5 per million, considerably higher than previous estimates.
• A recent Spanish study for WG incidence found it to be 2.95 per million.