AUTOIMMUNE VASCULITIS (AV) & WEGENER'S GRANULOMATOSIS  (WG)

Rare autoimmune diseases that  Inflame blood vessels       Last update 26 August 2008
This web page courtesy of EXODUS WEB HOSTING

Most of the files that formerly had to be viewed by downloading in Word format have been converted to .html files.

AS OF 1 JANUARY 2006, THE FORMER WEGENER'S GRANULOMATOSIS ASSOCIATION BECAME THE VASCULITIS FOUNDATION
THE MAILING ADDRESS, EMAIL ADDRESS, AND WEB PAGE ADDESSES HAVE CHANGED SEE SEC. 3 BELOW
THE PHONE and FAX NUMBERS HAVE REMAINED UNCHANGED.



CLICK HERE for a list of links and files available from this web page.

                        INTRODUCTION

1 - Sources Used For This Web Page
2 - Questions About Vasculitis?
3 - Feeling All Alone? - Contact Others  (Click here for email guidelines)
4 - You Need An Advocate  
5 - Records You Need 

                    ABOUT VASCULITIDES

6 - About Vasculitides (plural of vasculitis)
7 - Things to ask about Vasculitis  
8 - Life-long Follow-Up   
9 - Classifying Autoimmune Vasculitides  - Table 1, Figure 1, Figure 1a
10 - Rescue Therapies During Crisis

                 ABOUT WEGENER'S GRANULOMATOSIS

11 - Finding Information about WG
12 - Early Signs of WG
13 - An Overview of WG - Figure 2
14 - Description of WG  - Table 2
15 - Prognosis for WG Patients
16 - WG Symptoms at the Start
17 - Damaged Organs after Remission - Table 3
18 - Disease-Related Permanent Morbidity - Table 4

                GETTING DIAGNOSED WITH VASCULITIS

19 - Finding A Physician
20 - Talking With Physicians
21 - Diagnostic and Baseline Medical Tests - Table 5, Figures 3 & 4

                CONVENTIONAL TREATMENTS AND OPTIONS FOR SOME AVs

22 - Precautions & Treatment - Table 9
23 - Prevention of  Relapse
24 -  Modified Treatment
25 - Cytoxan and Prednisone
26 -  Alternatives to Cytoxan and Prednisone (Link to Table 7)
27 - Cytoxan and Glucocorticoid Side Effects

                NEWER TREATMENTS

28 - Cloned Antibody Experience
29 - Newer "Biological" Treatments  (Link to Table 8)


                DURING TREATMENT


30 - Body Changes
31 -  Osteopenia & Osteoporosis
32 - Peripheral Neuropathy (PN)
33 – Ulcerations
34 - Avascular Necrosis (AVN)
35 - Subglottal & Bronchial Stenosis - Table 6
36 - Nasal Health (Crusting and Irrigation) & Dental Health
37A - Pregnancy  and Vasculitis
37B - Diet Considerations


                SOURCES OF MEDICAL INFORMATION

38 - Finding Medical Information
39 - National Institutes of Health
40 - Web Pages Related to AV & WG

                MISCELLANEOUS ITEMS

41 - Abbreviations & Acronyms
42  - Slide presentations -  Download
 
                    TABLES
                                                                                  
#1- CLASSIFICATION OF AUTOIMMUNE VASCULITIDES AND SIMILAR DISEASES
#2 - SYMPTOMS AT ONSET OF WG
#3 - DAMAGES AFTER REMISSION                                                                          
#4 - LONG TERM MORBIDITIES                                                                                 
#5 - FREQUENCY OF POSITIVE ANTI-PR3 OR ANTI-MPO IN SOME AVs
#6 - SOME METHODS OF TREATING SUBGLOTTAL STENOSIS
#7 - MECHANISMS OF IMMUNOSUPPRESSIVES
#8 - MEDICATIONS IN DEVELOPMENT THAT MIGHTBE USEFUL TO TREAT SOME AVs
#9 - EQUIVALENT DOSAGES OF VARIOUS GLUCOCOTICOIDS
   
    FIGURES


#1 - VESSEL SIZES AND TYPICAL AV INVOLVMENTS
#2 - ORGANS MOST FREQUENTLY AFFECTED BY WG
#3 - GROSS VESSEL ABNORMALITIES
#4 - USING ANCA IN DIAGNOSING AV

CLICK HERE
TO DOWNLOAD A SLIDE SHOW, "PRIMER ON VASCULITIS".  AFTER OPENING THE FILE, CLICK ON "VIEW" AND SELECT "SLIDE SHOW".

CLICK HERE TO SEE A LIST OF QUESTIONS FOR PERSONS SUSPECTING OR HAVING AN AUTOIMMUNE VASCULITIS.


CLICK HERE TO VIEW A LIST OF SLIDE PRESENTATIONS ON VASCULITIS THAT CAN BE DOWNLOADED.



INTRODUCTION TO AUTOIMMUNE VASCULITIS

1 - SOURCES OF THIS WEB PAGE

2 - QUESTIONS ABOUT YOUR HEALTH AND VASCULITIS?

3 - FEELING ALL ALONE? 

VASCULITIS FOUNDATION (formerly the Wegener's Granulomatosis Association)
P. O. Box 28660
Kansas City, Missouri  64188-8660                                             
Toll free 1-800-277-9474
Fax/phone 1-816-848-4444
E-mail vf@vasculitisfoundation.org
VF (WGA) web page - http://www.vasculitisfoundation.org

(Click here to return to Index)
4 - WHY YOU NEED AN ADVOCATE

5 - RECORDS YOU NEED

(Click here to return to Index)

ABOUT VASCULITIS
6 - ABOUT VASCULITIDES (plural of vasculitis, vas-cu-lit'-i-deez, )
7 - THINGS TO ASK ABOUT VASCULITIDES
(Click here to return to Index)
8 - LIFE-LONG FOLLOW UP

(Click here to return to Index)

9 - CLASSIFYING AUTOIMMUNE VASCULITIDES (AVs) and somewhat similar diseases. 

TABLE 1 - CLASSIFICATION OF AUTOIMMUNE VASCULITIDES AND SIMILAR DISEASES

[Not generated by a medical professional]


PREDOMINANTLY AFFECTING LARGE- and MEDIUM-SIZED BLOOD VESSELS

Giant Cell Arteritis (GCA)                       Takayasu's Arteritis (TA)          Temporal Arteritis (GCA)

PREDOMINANTLY AFFECTING MEDIUM-SIZED BLOOD VESSELS

Polyarteritis Nodosa (PAN)                     Kawasaki's Disease (KD)          Primary CNS Vasculitis  (CNSV)
Thromboangiitis obliterans (TO?)           Buerger's Disease (BD)      

PREDOMINANTLY AFFECTING MEDIUM- and SMALL-SIZED BLOOD VESSELS - Usually ANCA positive

Churg-Strauss Syndrome (CSS)      Wegener's Granulomatosis (WG)      # Microscopic Polyangiitis (MPA
# Sometimes identified as microscopic polyarteritis

PREDOMINANTLY AFFECTING SMALL-SIZED BLOOD VESSELS - ANCA negative

Henoch-Schoenlein Purpura (HSP)      Leukocytoclastic Vasculitis (LV)     Lymphomatoid granulomatosis (LG?)
##  Goodpasture's disease (Anti-GBM)  Essential cryoglobulinemic vasculitis (LV)    

DISEASES THAT MAY BE AUTOIMMUNE VASCULITIDES OR HAVE SOME SYMPTOMS SIMILAR TO WG

Hypersensitivity Vasculitis (HV)             Rheumatoid Vasculitis (RV)         Relapsing Polychondritis
Buerger's Disease (BD)         Polymyalgia Rheumatica (PR)    Central Nervous System Vasculitis (CNSV)

OTHER AUTOIMMUNE DISEASES THAT MAY HAVE SYMPTOMS SIMILAR TO WG OR
ARE PERHAPS SUPERSEDED TERMINOLOGY


Necrotizing Respiratory Granulomatosis (NRG?)     Pathergic Granulomatosis (PG?)      Lethal Midline Granuloma (LMG?)
# Alpha-1 Antitrypsin Deficiency (AATD)     Systemic Lupus Erythematosis (SLE)
# Causes necrotizing respiratory vasculitis





FIGURE 1- VESSEL SIZES AND TYPICAL AV INVOLVMENTS




FIGURE 1A - PRESENTATION OF VASCULITIDES VERSUS VESSEL SIZES

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10 - RESCUE THERAPIES FOR AV PATIENTS IN CRISIS

(Click here to return to Index)

ABOUT WEGENER'S GRANULOMATOSIS (WG)

11 - FINDING INFORMATION ABOUT WG

12 -  EARLY SIGNS OF WG
CLINICAL FEATURES OF WEGENER'S GRANULOMATOSIS
 
  Ear/nose/throat/mouth   Nasal septum perforation, saddle-nose deformity, conductive or sensorineural hearing loss,
  subglottic stenosis, strawberry gums, oral ulcers
  Eye   Orbital pseudotumor, conjunctivitis, keratitis (risk of corneal melt), anterior uveitis, scleritis, episcleritis
  Lung   Nodules, infiltrates, cavitary lesions, alveolar hemorrhage, bronchial lesions
  Heart   Occasional valvular lesions, pericarditis
  Kidney   Segmental necrotizing glomerulonephritis, renal failure requiring hemodialysis
  Skin   Vesicular, palpable purpuric, ulcerative, or hemorrhagic lesions
  Nervous system
  Sensory or motor mononeuritis multiplex, aseptic meningitis

13 - AN OVERVIEW OF WG

(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

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14 -  DESCRIPTION OF WG