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22 Cards in this Set

  • Front
  • Back
Definition of vasculitis
inflamm destruc of vessels (usually arteries) causing ischemia, dysfunc or embolism through vessel narrowing or aneurysm formation.
Which Ig class did she mention can be present in vessels?
IgA
Skin clues of vasculitis
palpable purpura
Ulcer - often with defined or pointed edges
Nodules (tender under the skin)
ischemic digits
livido reticularis (net-like pattern under the skin) or reticulated purpura (purpura with points)
How to differentiate extravasated blood from dilation and stasis
diascopy - apply pressure and observe pressure changes

Hemorrhagic lesions and nonvascular lesions do not blanch; inflammatory lesions do
Vessels that can be affected
Aorta, large/med artery, small art, arteriole, capillary, venule, vein, vena cava.
What do "small vessels" include?
post-capillary venules, capillaries and arterioles.
Which infection did she specifically talk about that can cause vasculitis?
Hep B - often causes aneurysms.

Coronary arteries, mononeuritis.
Reasons to consider dx of vasculitis
Skin lesions (espec palpable purpura, stellate or punched out ulcers)

glomerulonephritis - rbc casts or blood/prtein in the urine.

mononeuritis multiplex - infarction of a nerve due to inflammation of vaso vasorum.

disease of the old in young

systemic disease without infection or malignancy

multisystem illness in people who aren't too old.
Primary vs. secondary
primary - the etiologic agent/antigen is unknown.

Secondary - due to another CT disease, infection (hep B or C, parvo, herpes, subacute bacterial endocarditis)

drug induced

malignancy (usually the liquid ones)

cryoglobulins - cold precipitating proteins

cardiac myxoma.
etiology of idiopathic vasculitis
immune complexes in vascular wall and inflammation results.

Usually due to adhesion molecules in vasc bed and turbulence of blood flow and hydrostatic pressure.

can be provoked by infections, exposures and genetic predisposition
e.g. wegener's and CTLA polymorphism or giant cell arteritis with chlamydia.
Wegener's granulomatosis triad
vasculitis, extravasc granuloma in UR and LRespiratory tract and glomerulonephritis.

Associated with ANCA antibodies.
Lab tests with vasculitis
usually nonspecific but the one exception is peri-nuclear anti-neutrophil antibody test (pANCA) or cytoplasmicANCA.
ANCA
autoantibodies directed at the enzymes and protein in the granules or neutrophils.

pattern and specificity of ab binding is important.

operatory dependent - the threshold for "positive" is hazy. ELISA can be done to get around this.

False positives are common (CTDisorders, infections, IBD, drugs, autoimmune...)

THIS IS THE ONLY SPECIFIC TEST FOR VASCULITIS AND IT IS NOT VERY SPECIFIC OR SENSITIVE!!! (must have suspicion before ordering this)
cANCA antigen
serine proteinase 3 (PR3)
pANCA antigen
myeloperoxidase (MPO) usually.
ANCA positive vasculities - incidence and presentation
fairly common and common presentation of pulmonary-renal syndrome.
giant cell arteritis or temporal arteritis - dx
BIOPSY!!! This is the only thing that tells us what the vessel wall is like.

choose newly symptomatic tissue. take a big chunk because lesions are intermittent.
when to do angiography to dx
when biopsy is unavailable...

and be wary of dx based on a lab test alone.
things that can mimic vasculitis
vasculopathy - processes that cause vessel obstruction, narrowing or aneurysm that is non-inflammatory.

Cholesterol emboli
SBE
Antiphospholipid antibodies
Disseminated intravascular coagulation
Ergot (vasoconstrictor for migraines) and sympathomimetic drugs
Vasospasm, seen especially in the CNS.
Calciphylaxis (deposition of calcium in vessel wall)
Amyloidosis
Fibromuscular dysplasia
Tx of vasculitis
Remove precipitants

Treat inflammation (corticosteroids)

Treat damaged endothelium and complications like hypertension (heparin or coumadin)

Monitor for active inflammation vs ischemic sequella

Move to lowest dose of immunosuppression that controls inflammation
the last thing to worry about is...
the TYPE of vasculitis your pt has.
you dont need to knnow...
specifics about specific diseases (ignore this part in the notes)