AUTOIMMUNE VASCULITIS
- Last update 11 Feb 2010
- Links to on-line information from professional medical sources.
- Click on a name or link to see the summary information.
- Click here to see more detailed information on WG.
- Click here for a listing of selected AV and medical links
Note 1 - Cutaneous PAN is a milder disease characterized by periodic exacerbations and often associated with streptoccoccal infection.VACULITIS FOUNDATION MAYO CLINICJOHNS HOPKINSCLEVELAND CLINICBehcet's disease
aka Behcet's syndrome (BS)Buerger's disease (BD) Central Nervous System (CNSV) Churg-Strauss Syndrome (CSS) Cryoglobulinemia (CRYO) Giant cell arteritis (GCA)
aka temporal arteritis (TA)Henoch-Schonlein purpura (HSP) Hypersensitivity vasculitis
Kawasaki disease (KD) Microscopic polyangiitis (MPA) Polyarteritis nodosa (PAN) NOTE 1 Polymyalgia Rheumatica (PR) Rheumatoid vasculitis (RV) Takayasu's arteritis (TAK) Wegener's granulomatosis (WG)
- Blood vessel size
- Usually large and medium
- Giant Cell Arteritis (GCA), also known as Temporal Arteritis (TA)
- Takayasu's Arteritis (TAK)
- Usually medium
- Buerger's Disease (BD)
- CNS Vasculitis (CNSV)
- Kawasaki's Disease (KD)
- Polyarteritis Nodosa (PAN)
- Usually medium and small
- Usually ANCA positive
- Churg-Strauss Syndrome (ANCA may be negative if no lung involvement)
- Microscopic Polyangiitis (MPA, aka microscopic polyarteritis]
- Wegener's Granulomatosis (WG)
- Usually ANCA positive
- Usually small
- Usually ANCA negative
- Cryoglobulinemic vasculitis (LV) [Leukccytoclastic?]
- Henoch-Schoenlein Purpura (HSP)
- Polymyalgia Rheumatica (PR)
- Rheumatoid Vasculitis (RV) [may have atypical positive ANCA]
- Usually ANCA negative
- All sizes
- Behcet's syndrome (including veins)
- Hypersensitivity Vasculitis (HV)
- An early 2009 report suggests that Cutaneous Polyarteritis Nodosa (CPN) does not progress to polyarteritis nodosa (PN), and introduced new drafts for CPN and PN diagnostic criteria.
- Usually large and medium
FIGURE 2- VESSEL SIZES AND TYPICAL AV INVOLVMENTS
 
FIGURE 3 - VASCULITIDES VERSUS VESSEL SIZES
- Getting started. This section is an overview about diagnosis.
- Click here to go to the section,"GETTING DIAGNOSED" for more details.
- History - Have a summary history of major surgeries and illnesses readily available for all appointments.
Delays - Diagnosis is often delayed due to the general lack of experience and knowledge about autoimmune vasculitides in the medical community and general population.- It takes persistence and research to find a physician with the expertise and experience to be capable of correctly diagnosing autoimmune vasculitides.
- Expedite diagnosis aggressively. Serious or even mortal damage can be done, sometimes quickly. If you don't get satisfactory answers from your initial physician, get a new physician.
- Don't hesitate to insist your physician consult with a specialist listed in the Vasculitis Foundation consultants as listed at http://www.vasculitisfoundation.org/node/44
- Expertise - If one can't locate the needed expertise locally, then one must resort to a major medical center or teaching hospital.
- For the latter, 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. Always ask the "doctor", his/her status: intern, resident, fellow, department head, so you know the level of training.
- Referrals - Don't hesitate to request referral to a major medical center, preferably one that specializes in autoimmune vasculitis.
- Some notable Vasculitis Centers are:
- The Cleveland Clinic Center for Vasculitis Research and Treatment at http://www.clevelandclinic.org/arthritis/vasculitis/default.htm
- The Johns Hopkins Vasculitis Center at http://vasculitis.med.jhu.edu/
- National Jewish Medical and Research Center (Denver, Colorado) at http://www.njc.org/
- The University of Alabama at Birmingham at http://www.health.uab.edu/default.aspx?pid=82347
- Mayo Clinic in Rochester, MN at http://www.mayo.edu/
- Boston University - Vasculitis Center (Boston, MA) at http://rarediseasesnetwork.epi.usf.edu/vcrc/centers/boston.htm
- Mount Sinai Hospital (Toronto, Canada at https://www.mtsinai.on.ca/rmcad/contact.htm
- For a more complete list, see http://www.wegenersgranulomatosis.net/Vasculitis-URLS.html - 11._MAJOR_MEDICAL_CENTERS
- Some notable Vasculitis Centers are:
- Persistence - Keep trying until you get good answers and effective treatment.
- Women especially are often discouraged by being told they are imagining or exaggerating their ailments.
- This is often not true. Women know their bodies and know when something is not right.
- It's vital to keep looking until you get positive answers and effective treatment.
- Ask to be on cancellation waiting lists to get earlier appointments.
- If in serious trouble, go to an Emergency Room and hope they have the expertise needed. Explain you have or suspect an autoimmune vasculitis, but give specific symptoms.
- Women especially are often discouraged by being told they are imagining or exaggerating their ailments.
- AARDA - Click here for more information on getting a diagnosis of an autoimmune disease adapted from an article from the AARDA publication, InFocus, Vol.10, No. 22, June 002.
- Clinical trials - For persons with no or limited medical insurance, the National Institutes of Health had on-going clinical studies.
- If an AV patient qualified, most medical expenses associated with the clinical trial are paid while in the study.
- Current Clinical studies funded by the U.S. NIH can be found at http://clinicaltrials.gov/ct/screen/AdvancedSearch
- Many clinical trials are listed at http://www.centerwatch.com/
- European clinical studies are listed at http://www.med.cam.ac.uk/nephrology/research/vasculitis.html
- Differential Diagnoses
- The initial clinical appearances of various other diseases can mimic those of systemic vasculitis: bacterial endocarditis, Lyme disease, hypertensive arteriopathy, effects of vasoconstrictive drugs (e.g. ergotism), thoracic outlet syndrome, cholesterole embolism, and right atrial myxoma.
TABLE 1 - DIFFERENTIAL DIAGNOSES OF VASCULITIS
Original at http://doctorsforadults.com/essentials/cdim_ch90_wed01.pdf]
Click here to view a different table of differential diagnoses
FIGURE 4 - DIAGNOSING SMALL VESSEL VASCULITIS
- Secondary vasculitis
- Secondary vasculitis can be the result of many non-vasculitic conditions.
- Cutaneous pseudovasculitis represents a heterogeneous collection of disorders that are capable of simulating cutaneous vasculitis.
- Skin biopsy is a crucial step in differentiating pseudovasculitis from authentic vasculitis; absence of histologic evidence of vasculitis, particularly after multiple biopsies, should direct evaluation and diagnosis towards pseudovasculitis.
- These can be broadly classified into diseases that produce hemorrhage (petechiae, purpura, and ecchymoses) or vessel occlusion with resultant livedo, cyanosis, ulcers, digital necrosis, and/or gangrene.
- Overlap is not uncommon, but if present, one mechanism dominates.
- Hemorrhagic pseudovasculitis is due to vessel wall dysfunction (incompetence), which can be related to diverse factors that include vessel wall deposition of metabolic substances (amyloid, calcium), nutritional deficiencies (scurvy), nonvasculitic inflammatory purpura (pigmented purpuric dermatitis, arthropod, viral and drug reactions), degeneration of the vessel wall and supporting stroma (senile/solar purpura), direct vessel wall invasion of infective organisms, coagulation-fibrinolytic disorders (eg, thrombocytopenia), and vessel wall trauma.
- Cyanotic-infarctive pseudovasculitis is due vaso-occlusion by emboli, thrombi, or fibrointimal hyperplasia (endarteritis obliterans) and includes varied conditions such as purpura fulminans, Coumadin necrosis, antiphospholipid antibody syndrome, cardiac myxoma, cholesterol embolization, calciphylaxis, and radiation arteritis.
- Delayed and inappropriate diagnosis of pseudovasculitis leads to incorrect management and exposure to potentially deleterious treatment modalities such as corticosteroids and cytotoxic agents. The diagnosis of a pseudovasculitic disorder requires a high index of suspicion and should always be part of the differential diagnosis of vasculitis.
- Click here to view a table of pseudovasculitis causes
- Pregnancy & Vasculitis
- Pregnancy and vasculitis are a serious combination. Special precautions are needed to optimize the health of the mother and the fetus.
- Click Here to view a file on pregnancy and vasculitis.
- Similar Diseases
- Diseases that may be AVs or have symptoms similar to an AV:
- Erythema nodosum
- Eosinophilic gastroenteritis (EG)
- Goodpasture's disease (Anti-GBM) [strictly speaking, not vasculitis]
- Lymphomatoid granulomatosis (LG?)
- Thromboangiitis obliterans
- Other autoimmune diseases that may have symptoms similar to an AV:
- Alpha-1 Antitrypsin Deficiency (AATD) [Causes necrotizing respiratory vasculitis]
- Goodpasture's disease (Anti-GBM) [strictly speaking, not vasculitis]
- Lethal Midline Granuloma (LMG?)
- Necrotizing Respiratory Granulomatosis (NRG?)
- Pathergic Granulomatosis (PG?)
- Relapsing Polychondritis
- Systemic Lupus Erythematosis (SLE)
- Diseases that may be AVs or have symptoms similar to an AV:
- Vision Involvements
- Click Here to view in formation on inflammations of the eye
- Blood flow to muscles and nerves of the eye are sometimes affected.
THIS SITE
- Intent
- To assist vasculitis patients in getting early diagnoses, effective treatments, and to advise of patient, organization, and scientific resources concerning vasculitis.
- Sources Used
- The following information is derived from a variety of sources over some ten+ 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 on vasculitis. Some is from autoimmune vasculitis patients and carers, some from newsgroups, internet web pages, etc. that also deal with vasculitis.
- The compiler has attempted to use only recent valid medical information regarding vasculitis, but cannot guarantee the validity nor the currency in every case
- Limitations
- 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 professional medical publication.
- Limits to this web page concerning vasculitis:
- The author/compiler/editor of this web page and related pages has had NO medical training.
- Only autoimmune vasculitides will be considered, not hypersensitivity vasculitis nor vasculitis as a result of an allergic reaction to medication or vaccine..
- Most sections apply to most autoimmune vasculitides.
- One refers specifically to Wegener’s granulomatosis.
- Terminology
- Some abbreviations and equivalencies are:
- Hereafter, “autoimmune vasculitis” may be abbreviated “AV” or “AVs” for plural.
- The term “Prednisone” is sometimes used where it or a similar glucocorticoid might be prescribed to treat vasculitis.
- “Immunosuppressives” used to treat vasculitis are sometimes abbreviated “ISs”.
- Some abbreviations and equivalencies are:
- Updates
- This update was on June 1, 2009 and is a complete rewrite of the former web page at http://www.wegenersgranulomatosis.net that also dealt with vasculitis.
- Files & links – Vasculitis related.
- Tables & Figures – Vasculitis related
- Disclaimer
- ALL MEDICAL QUESTIONS, SYMPTOMS, CONCERNS AND PROBLEMS SHOULD BE DIRECTED TO APROPRIATE LICENSED MEDICAL PROFESSIONALS.
- The writer/editor/compiler does not vouch for the accuracy, completeness, nor applicability of the information included on this site to any person, whether a vasculitis patient or otherwise.
Please forward comments and corrections to blades49456@sbcglobal.net
