Vasculitis Flashcards
clues to vasculitis
skin- palpable purpura, ulcers, nodules, ischemic digits, livido reticularis GNitis mononeuritis multiplex dz of the old in the young (like CAD) systemic dx w/o infection or malig
vasculitis definition
inflammatory destruction of vessels causing ischemia, dysfunction or embolism through vessel narrowing or embolism
causes of primary vasculitis
idiopathic
causes of secondary vasculitis
occurs with CT disease, infection (hep C, B, parvo, zoster, SBE), drugs, malignancy
what lab tests are specific for vasculitis
ANCA
only way to confirm vasculitis diagnosis
biopsy
types of small vessel vasculitises
henoch-schoenlen purpura
microscopic polyarteritis/polyangitis
wegener’s granulomatosis
churg-strassaiul syndrome
Henoch-Schoenlen Purpura
skin rash in areas of high hydrostatic pressure (legs, arms)
purpura dont blanch
blood diarrhea (vasculitis of bowel lining)
GNitis
arthralgias
bx of Henoch Schoenelen Purpura
neutrophilic, leukocytoclastic vasculitis
IGA
polyarteritis nodosa is almost always caused by
hepatitis B infection
polyarteritis nodosa symptoms
renal artery involvement (HTN) gut ischemia deep cutaneous ulcers** peripheral neuropathy mononeuritis
what else can present with polyarteris nodosa
weight loss, fever, lack of localizing signs
Kawaski’s disease is…
Polyarteris nodosa in children <6
symptosm of kawaski’s dz
inflammation of oral mucous membranes, lympahdenopathy, rash, irritability
complication in kawaski’s
coronary arteriris
main cause of vasculitis in adults over 55
giant cell arteritis
what is the patho phys of GCA
mononuclear cell infiltrate destroys elastic lamina of muscular arteries–> become tender, non-elastic, and non-pulsatile
what does GCA primarily affect
extracranial elastic arteries causing..
facial pain, visual loss from ischemic optic neuropathy, painful proximal muscular aching (PR)
catchphrase of giant cell arteritis
“pain putting a hat on”
tx of GCA
prednisone (excellent response)
Takayasu’s aoritis
related to GCA, but more common in younger pts, asians, F>M
pathophys of Takayasu’s
affected vessels are more proximal–hallmark is dz is aorta and its branches
initial vs late phase of takayasu’s
initial- inflam! fever, malaise, weight loss, ischemic symptoms
later- progressive vascular occlusions w/o signs of systemic inflammation
microscopic polyarteritis/polyangiitis
wegner’s like in presentation–typically involves skin, kidneys, lungs w/ diffuse alveolar hemorrhage syndrome
no extravascular granulomas
wegner’s classic traid
vasculitis and extravascular granulomatous inflam in
- upper airways (nose, sinuses, ears)
- lower airways (lungs, trachea)
- kidney (pauci-immune gnitis)
what else may the pt present with in wegner’s
systemic signs
epistaxis
shortness of breath
evidence of GN
ancas in mPAN vs wegener’s
mPAN- panca
Wegener’s-canca
Churg Strauss syndrome
another wegner’s like dz in patients with a hx of asthma or atrophy
halmarks of churg strauss
pulmonary infiltrates
cardiac and neurologic features w/ blood eosinophilia
primary vs secondary raynaud’s
primary- disease of young
secondary- adults with other dz (systemic sclerosis, CREST, SLE, DM, smoking)
what is secondary raynaud’s due to
exagerated vasomotor response to cold or stress
tx raynaud’s
- avoid triggers (cold- gloves, smoking, stress, sympathomimetics, coffee)
- Ca channel blockers (nifedipine)
- sidenafil
Behcet’s
peculiar involving veins as well as arteries–associated with intensely painful oral and genital ulcers, thrombophelbitis, retinal vasculitis, and anterior eye diz, encephalitis and rash
cryoglobulinemic vasculitis
cold ppt antbodies causing a small vessel vasculitis inducing GN most strongly a/w hep B
buerger disease
aka thromboangitis obliterans
- obliterative arterial and venous disease of the hand and feet felt by some of the vasculopathy rather than a true vasculitis
- ->causes digit loss and closely related to cigarette smoking
