Transcript VASCULITIS (vas cue lie tis) - Vasculitis Patient Information

VASCULITIS
(vas que LY tis)
Inflammation of blood vessels
September 5, 2006
THIS PRESENTATION
PURPOSES


PATIENT SUPPORT INFORMATION
INCREASED PUBLIC AWARENESS
45 SLIDES - ONLY AUTOIMMUNE VASCULITIS
3 SLIDES - ON-LINE SUPPORT GROUPS
12 SLIDES - E-MAIL SUPPORT GROUPS
VASCULITIS AWARENESS: SEPT 24-30, 2006
KINDS OF VASCULITIS
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HYPER-SENSITIVIE
Body responds to allergens - multiple factors
Exposure to medications
Genetic Predisposition
Environmental causes
Usually stops when allergen is removed
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INFECTIOUS
Various viruses and bacteria
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AUTOIMMUNE
Malfunction of immune system - multiple factors
Exposure to medications
Genetic predisposition
Environmental exposures
Often chronic (permanent change in immune system)
WHAT TO DO FIRST?
If you have been diagnosed with or suspect
vasculitis, for early patient support:
VASCULITIS FOUNDATION
Kansas City, MO
http://www.vasculitisfoundation.org
1-800-277-9474 U.S.
816-436-8211 Foreign & Fax
INTRODUCTION TO
AUTOIMMUNE VASCULITIS
(Abbreviated as “AV”)
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NEVER HEARD OF AUTOIMMUNE
VASCULITIS ?
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WELL, THAT’S BECAUSE IT’S RARE
HOW RARE?
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INCIDENCE OF AVs RANGES FROM
ABOUT 1 IN 10 THOUSAND TO ABOUT 1
IN 50 THOUSAND PERSONS
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YOU MAY KNOW SOMEONE WHO HAS
AUTOIMMUNE VASCULITIS
WHAT IS AUTOIMMUNE?
THE IMMUNE SYSTEM CONSISTS OF A WIDE
VARIETY OF CELLS AND ANTIBODIES THAT
GUARD AGAINST INFECTION
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AUTOIMMUNITY IS A MALFUNCTIONING
IMMUNE SYSTEM
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THE BODY’S IMMUNE SYSTEM MISTAKES
THE BODY’S TISSUES FOR BACTERIAL OR
VIRAL INVADERS AND ATTACKS IT’S OWN
TISSUES!
COMMON AUTOIMMUNE DISEASES
 RHEUMATOID ARTHRITIS
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LUPUS ERYTHEMATOSIS
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HYPOTHYROIDISM
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AUTOIMMUNE LIVER DISEASE
THESE ARE NOT VASCULITIS
[Although vasculitis can happen in RA and SLE]
WHAT IS VASCULITIS?
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INFLAMMATION OF THE WALLS OF BLOOD
VESSELS
CAN BE ANYWHERE IN THE BODY
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DAMAGES VESSELS AND ADJACENT
TISSUES

CAN BE PRIMARY OR SECONDARY TO
ANOTHER MEDICAL CONDITION
WHAT BLOOD VESSELS
ARE AFFECTED?
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IT VARIES GREATLY, BUT ANY BLOOD
VESSEL CAN BE AFFECTED:
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ARTERIES
ATERIOLES
CAPILLARIES
VENULES
VEINS
CAN DAMAGE VIRTUALLY ANY ORGAN
OR TISSUE
VESSEL SIZE & TYPE OF AV
THINGS TO KNOW ABOUT AVs

AUTOIMMUNE VASCULITIS OFTEN
GOES UNDIAGNOSISED FOR
MONTHS AND YEARS
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EVENTUALLY A CRISIS FORCES
PATIENTS AND PHYSICIANS TO SEEK
MORE EXPERT HELP
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AUTOIMMUNE VASCULITIS IS NOT
CONTAGIOUS
MORE ABOUT AUTOIMMUNE
VASCULITIS

AUTOIMMUNE VASCULITIS IS NOT
DIRECTLY INHERITABLE
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THE TENDENCY TO DEVELOP AN
AUTOIMMUNE DISEASE IS INHERITABLE
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THERE ARE ABOUT 15 KINDS OF AVs
NOTE - PLURAL OF VASCULITIS IS VASCULITIDES
“VAS-QUE-LIT-I-DEEZ”
EVEN MORE ABOUT
AUTOIMMUNE VASCULITIS

CAN BE LOCAL OR SYSTEMIC
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SYSTEMIC CAN INVOLVE MANY
ORGANS AND TISSUES.
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UNTREATED LOCAL AV MAY
BECOME SYSTEMIC
NAMES OF AUTOIMMUNE
VASCULITIDES
Behcet’s Disease
 Buerger’s Disease
 Central Nervous System Vasculitis
(CNSV)
 Churg-Strauss Syndrome (CSS)
 Cryoglobulinemic vasculitis
 Giant Cell Arteritis (GCA)
 Henock-Schonlein Purpura (HSP)
 Hypersensitivity Vasculitis
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(MORE)
MORE NAMES OF
AUTOIMMUNE VASCULITIDES
Kawasaki’s Disease (KD)
 Microscopic Polyangiitis (MPA)
 Polyarteritis nodosa (PAN)
 Polymyalgia rheumatica (PR)
 Rheumatoid vasculitis (RV)
 Takayasu’s arteritis (TAK)
 Wegener’s granulomatosis (WG}
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ABOUT AUTOIMMUNE VASCULITIS

MOST UNTREATED AUTOIMMUNE
VASCULITIS IS FATAL SOONER OR
LATER

VASCULITIS CAN ALMOST ALWAYS BE
CONTROLLED (Remission)
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KEY TO CONTROL IS EARLY DIAGNOSIS
AUTOIMMUNE VASCULITIS CAUSES
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ENVIRONMENTAL EXPOSURES
Silica, Organic compounds, ?

GENETIC PREDISPOSITION
Family members with autoimmune diseases
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INFECTION
Bacterial, Viral, Parasitical ?
No clear proof yet although in some cases
hepatitis B and C are associated with
vasculitis
WHO GETS AUTOIMMUNE
VASCULITIS?
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INFANTS
CHILDREN
ADOLESCENTS
ADULTS
SENIORS
FEMALES
MALES
ANYONE CAN DEVELOP AN AV !
HOW DOES AV SHOW UP?

IN A VERY WIDE VARIETY OF
SYMPTOMS
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TWO PEOPLE WITH THE SAME
AUTOIMMUNE VASCULITIS MAY
HAVE SIGNIFICANTLY DIFFERENT
SYMPTOMS
ABOUT SYMPTOMS OF
AUTOIMMUNE VASCULITIS

AUTOIMMUNE VASCULITIDES
MIMIC OTHER MORE COMMON
DISEASES

RARELY ARE SYMPTOMS ALONE
ENOUGH TO DIAGNOSE AN AV
AUTOIMMUNE VASCULITIS SYMPTOMS
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RASH
FATIGUE
UNEXPLAINED FEVER
WEAKNESS
HEADACHE
UNEXPLAINED WEIGHT LOSS
PAIN IN MUSCLES &/OR JOINTS
(More)
MORE AV SYMPTOMS
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CHRONIC COUGH
BLOOD IN URINE
BLOODSHOT EYES
FLUID IN EARS
CHANGES IN MEMORY,
COORDINATION, BALANCE
CHANGES IN VISION
And … , and…. , and…..
COULD I HAVE VASCULITIS?

ANY TIME YOU GET SICK, IT IS
UNLIKELY YOU HAVE AN AV

AVs ARE RARE AND MIMIC MANY
OTHER ORDINARY DISEASES

CHANCES ARE YOU HAVE ONLY SOME
ORDINARY TREATABLE CONDITION
TESTS FOR
AUTOIMMUNE VASCULITIS?

MOST AUTOIMMUNE VASCULITIDES DON’T
HAVE SPECIFIC TESTS THAT CAN
DIAGNOSE THE AV WITHOUT DOUBT
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THERE ARE A VARIETY OF TESTS THAT
CAN INDICATE AN INFLAMMATORY
CONDITION
OFTEN BIOPSY IS REQUIRED
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SOME ANTIBODY TESTS CAN HELP
SOME RADIOGRAPHIC STUDIES CAN HELP
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ANGIOGRAPHY, MRID, CT SCANS
HOW IS AUTOIMMUNE VASCULITIS
DIAGNOSED?

DIAGNOSIS IS MOSTLY BY
ELIMINATING ALL OTHER
POSSIBLE CAUSES.

DIAGNOSIS CAN BE DIFFICULT,
EXPENSIVE AND LENGTHY
WHO DIAGNOSES
AUTOIMMUNE VASCULITIS?
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OFTEN A RHEUMATOLOGIST

CAN BE ANY PHYSICIAN, OR
MEDICAL PROFESSIONAL WHO
SUSPECTS AUTOIMMUNE
VASCULITIS
WHO TREATS
AUTOIMMUNE VASCULITIS?
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RHEUMATOLOGISTS
NEPHROLOGISTS
PULMONARY SPECIALISTS
NEUROLOGISTS
DERMATOLOGISTS
MANY OTHER SPECIALTIES
DEPENDING ON WHAT ORGANS
AND TISSUES ARE AFFECTED.
WHERE TO GET DIAGNOSED?

PHYSICIANS EXPERIENCED IN
DIAGNOSING AND TREATING
VASCULITIS

OFTEN AT MAJOR MEDICAL
CENTERS AND TEACHING
HOSPITALS.
HOW IS VASCULITIS TREATED?
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VIRTUALLY ALWAYS WITH PREDNISONE
OR SIMILAR GLUCOCORTICOIDS
 FREQUENTLY WITH STRONG DRUGS TO
SUPPRESS THE IMMUNE SYSTEM
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TREATMENT IS LENGTHY, MONTHS OR
YEARS

STOP MEDICATIONS THAT CAUSE
ALLERGIC & AUTOIMMUNE REACTIONS
WHAT IMMUNOSUPPRESSIVES?
CANCER DRUGS USED IN MUCH SMALLER
DOSAGES
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CYTOXAN
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METHOTREXATE
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CELLCEPT
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IMURAN
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LEUKERAN
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UNUSUALLY OTHER
IMMUNOSUPPRESSIVES
OTHER TREATMENT METHODS
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BLOOD PLASMA EXCHANGE CALLED
PLASMAPHERESIS THAT REMOVES
HARMFUL ANTIBODIES FROM BLOOD
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INTRAVENOUS IMMUNOCLOBULIN “G”
(IVIg) (INFREQUENTLY EXCEPT FOR KAWASAKI DISEASE)
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NEW “BIOLOGICAL” MEDS THAT
SELECTIVELY SUPPRESS SPECIFIC
PARTS OF THE IMMUNE SYSTEM
INFORMATION ON VASCULITIS
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VASCULITIS FOUNDATION IN KANSAS CITY, MO
1-800-277-9474 [email protected]
http://www.vasculitisfoundation.org

VASCULITIS CLINICAL RESEARCH CONSORTIUM
(VCRC)
1-617-414-2500 [email protected]
http://rarediseasesnetwork.epi.usf.edu/vcrc/index.htm
MORE INFORMATION
ON VASCULITIS
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Cleveland Clinic Center for Vasculitis
http://www.clevelandclinic.org/arthritis/vasculitis/default.htm
216-445-6996
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[email protected]
Johns Hopkins Vasculitis Center
http://vasculitis.med.jhu.edu/
410-550–6825
[email protected]
MORE INFORMATION
ON VASCULITIS
MAYO CLINIC - ROCHESTER MN
Dr. Ulrich Specks M.D.
507-284-8450
[email protected]
http://www.mayoclinic.org/pulmonary-rst/diseases.html
UNIVERSITY OF NORTH CAROLINA
Dr. Ronald Falk M.D.
[email protected]
Ph (919) 966-2561 x 224
Office 349 MacNider Hall
http://www.unckidneycenter.org/vasculitis_brochure.pdf
INTERNATIONAL CONTACTS FOR
AUTOIMMUNE VASCULITIDES
BRAZIL
ALEXANDRA VILLA-FORTE M.D.
Disciplina de Rhumatolgia, Departamento de
Mediciana, Escola Paulista de Medicina
Universidade Federal de Sco Paulo Brazil
E-mail: [email protected]
INTERNATIONAL CONTACTS (continued)
FRANCE
LOIC GUILLEVIN M.D.
Department of Internal Medicine
Hospital Cochin, University Paris 5
27 rue du Faubourg Saint Jacques, 75014
Paris, France
Phone: 33 158-411321
Email: [email protected]
(MORE)
INTERNATIONAL CONTACTS (continued)
GERMANY
WOLFGANG L GROSS M.D., PH.D.
Director, Dept. of Rheumatology,
University of Lübeck,
Clinic for Rheumatology,
Bad Bramstedt, Ratzeburger Allee 160,
Lübeck, 23538 Germany
Phone: 49-451-500-2368
Email: [email protected]
(MORE)
INTERNATIONAL CONTACTS (continued)
IRELAND
GEORGE MELLOTTE M.D.
St. James Hospital, James St.
Dublin 8, Ireland
Ph 3531-410-3000
[email protected]
http://webrheum.bham.ac.uk/vasculitis/
(MORE)
INTERNATIONAL CONTACTS (continued)
SPAIN
MARIA C. CID M.D.,
Department of Internal Medicine
Hospital Clinic – University of Barcelona
Villarroel 170, Barcelona, Spain 08036,
Phone: 34-93-227-9365,
Email: [email protected]
(MORE)
INTERNATIONAL CONTACTS (continued)
UNITED KINGDOM
DAVID JAYNE M.D.
The Vasculitis Service and Vasculitis Clinic
Box 118, Renal Unit,
Addenbrooke’s Hospital
Cambridge CB2 2QQ, United Kingdom
Phone: 44-1223-217259,
Fax: 44-1223-586797
Email: [email protected]
http://www.addenbrookes.nhs.uk/serv/clin/med/vasculitis1.html
(MORE)
INTERNATIONAL CLINICS AND
ORGANIZATIONS
European Vasculitis Study Group (EUVAS):
http://www.vasculitis.org
Groupe Français d'Etude des Vascularites:
http://www.vascularite.com/
International Network for the Study of Systemic
Vasculitides (INSSYS)
http://www2.ccf.org/inssys/
Rheuma-Klinik Bad Bramstedt
http://www.rheuma-zentrum.de
BOOKLETS/BROCHURES
University of North Carolina Brochure
“What Is Small-Vessel Vasculitis?”
http://www.unckidneycenter.org/vasculitis_brochure.pdf
Stuart Strange Trust, U.K. Booklet
“Living with Vasculitis”
http://webrheum.bham.ac.uk/Vasculitis/Booklet/LivingWithVasculitis.pdf
“Wegener’s Granulomatosis”
http://www.orpha.net/data/patho/GB/uk-WG.pdf
(All require Adobe Acrobat Reader)
(More)
MORE BOOKLETS/BROCHURES
“What you should know about
Wegener’s Granulomatosis”
http://www.stjames.ie/ClinicalInformation/WegenersGranulomatosis
Handbook/file,8398,en.PDF
“Wegener’s Granulomatosis”
http://www.vumc.nl/aig/onderwijs/ROIG/23112005/
ANCA%20vasculitisgross.pdf
(Both above require Adobe Acrobat Reader)
SOURCES FOR INFORMATION
ON VASCULITIS

NATIONAL LIBRARY OF MEDICINE
(gateway.nlm.nih.gov http://www.ncbi.nlm.nih.gov)
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ON-LINE MEDICAL PROFESSIONAL WEB PAGES
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HOSPITAL MEDICAL LIBRARIES
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ON-LINE EMAIL GROUPS AND MESSAGE
BOARDS
VASCULITIS PATIENT SUPPORT
ORGANIZATIONS
Vasculitis Foundation (All vasculitides)
http://www.vasculitisfoundation.org
Central Nervous System Vasculitis
Foundation Inc. (CNSV)
http://www.cnsvfinc.org/contact.php
Churg-Strauss Syndrome Assoc. (CSS)
http://www.cssassociation.org
(More)
VASCULITIS PATIENT SUPPORT
(continued)
Churg-Strauss Syndrome (CSS)
International Support Group
http://www.churg-strauss.com/
Giant Cell (Temporal) Arteritis (GCA)
http://nmrfoundation.blogspot.com/
Polyarteritis Nodosa (PAN)
http://www.pansupport.org/
(More)
VASCULITIS PATIENT
SUPPORT(continued)
Takayasu's Arteritis Association (TAK)
http://www.takayasus.com
Wegener’s Granulomatosis (WG)
http://vasculitisfoundation.org
End Patient Support Groups
SELECTED AUTOIMMUNE
E-MAIL GROUPS
Autoimmune Diseases E-mail Group
http://groups.yahoo.com/autoimmunediseases
Auto_immune_diseases E-mail Group
http://groups.yahoo.com/auto_immune_disease
Autoimmune Disorders E-mail Group
http://groups.yahoo.com/autoimmunedisorder
(More)
ARTERITIS E-MAIL GROUPS
Arteritis – Vasculitis Clinical Research Consortium
http://groups.yahoo.com/arteritis
Arteritis – Giant Cell / Temporal Arteritis E-mail Group
http://groups.yahoo.com/giantcellarteritis
Arteritis – Takayasu’s E-mail Group
http://groups.yahoo.com/Takayasus_arteritis
(More email groups)
CHURG-STRAUSS SYNDROME
E-MAIL GROUPS
Churg-Strauss Syndrome – CSSISG E-mail group
http://groups.yahoo.com/CSSISG
Churg-Strauss-Syndrome E-mail group
http://groups.yahoo.com/Churg-Strauss-Syndrome
Churg-Strauss_Syndrome E-mail group
http://groups.yahoo.com/Churg-Strauss_Syndrome
(More email groups)
HENOCH-SCHONLEIN PURPURA
E-MAIL GROUPS
Henoch-Schonlein Purpura (HSP)
http://groups.yahoo.com/Henoch_Schonlein
Henoch-Schonlein Purpura - Adults Only
http://groups.yahoo.com/HSP-Adults
(More email groups)
POLYARTERITIS NODOSA
E-MAIL GROUPS
Polyarteritis – Cutaneous PAN
http://groups.yahoo.com/cutaneouspan@yahoogro
Polyarteritis Nodosa
http://groups.yahoo.com/polyarteritisnodosa
Polyarteritis_nodosa
http://groups.yahoo.com/polyarteritis_nodosa
(More email groups)
VASCULITIS E-MAIL GROUPS
Vasculitis – CNSV
http://cnsvfinc.org/mailman/listinfo/whisperingangels
Vasculitis
http://groups.yahoo.com/vasculitis
Vasculitis Ed Center
http://groups.yahoo.com/vasculitis-ed-center
Vasculitis – Indian
http://groups.yahoo.com/irvas
(More email groups)
VASCULITIS E-MAIL GROUPS
(continued)
Vasculitis of the Central Nervous System)
http://groups.yahoo.com/cnsv
Vasculitis - Netherlands Vasculitis E-mail Group
http://groups.yahoo.com/Vasculitis-nl (Dutch language)
Vasculitis - Rheumatoid – E-mail Group
http://groups.yahoo.com/Rheumatoid_Vasculitis
Vasculitis- Savvy E-mail Group
http://groups.yahoo.com/savvy
(More email groups)
VASCULITIS E-MAIL GROUPS
(continued)
Vasculitis Support E-mail Group
http://groups.yahoo.com/vasculitissupport
Vasculitis Support Group –E-mail Group
http://groups.yahoo.com/vasculitissupportgroup
Vasculitis – Vasculitissurvivors E-mail Group
http://groups.yahoo.com/vasculitissurvivors
(More email groups)
WEGENER’S GRANULOMATOSIS
E-MAIL GROUPS
Wegeners E-mail Group
http://groups.yahoo.com/wegeners
Wegener's Granulomatosis - wgdiscussion
http://groups.yahoo.com/[email protected]
Wegener's Granulomatosis - European Group
http://groups.yahoo.com/wegenersdisease
Wegener's Granulomatosis – Fight Wegener’s
http://groups.yahoo.com/fightwegeners
(More email groups)
WEGENER’S GRANULOMATOSIS
E-MAIL GROUPS (continued)
WG - International Rare Disease Network
http://groups.yahoo.com/wegener's_granulomatosis
Wegener's Granulomatosis - Parents
http://groups.yahoo.com/parents4wegeners
Wegener's Granulomatosis - Australia
http://groups.yahoo.com/wegenersgranulomatosisaus
tralia
Wegener's Granulomatosis - Teens Only
http://groups.yahoo.com/wegeners-teens-only
(More email groups)
WEGENER’S GRANULOMATOSIS
E-MAIL GROUPS - (continued)
Wegener’s Granulomatosis –
South Carolina Support Group
http://groups.yahoo.com/Sc_wg_support_group
Wegener’s Granulomatosis - Weight Loss
http://groups.yahoo.com/Weggies4weightloss
AUTOIMMUNE VASCULITIS
PRESENTATION
THE END