Vasculitis Syndromes Flashcards
what are the 3 vasculitis syndromes we are learning about
Polyarteritis nodosa (PAN)
Giant cell arteritis/Temporal Arteritis
Takaysu’s Arteritis
what happens in a vasculitis
inflammation and damage to the BVs
the vessel lumen is usually compromised and this is associated with ischemia of the tissues supplied by the involved vessel
how is vasculitis syndrome classified
as affecting the small, med, or lg vessels
what is the accepted theory?
the deposition of immune complexes in the vessel walls results in compromise of the vessel lumen with ischemic changes in the tissues supplied by the involved vessels
what size vessels does PAN affect
small and medium
involvement with which arteries is common in PAN
renal arteries
visceral arteries
***characteristic
what two diseases can PAN be associated with
hairy cell leukemia
significant HTN
where do the lesions usually affect the arteries in PAN
at the bifurcation and branching of the arteries
which type of cell infiltrates the vessel wall and perivascular areas, resulting in intimal proliferation and degeneration of the vessel wall (PAN)
polymorphonuclear neutrophils
what comes along with necrosis of the vessel (PAN)
compromise of the lumen, thrombosis, or infarction of the tissue
as the lesions heal, that is deposited in the vessel which may cause further occlusion of the lumen? PAN
collagen deposition
what sort of dilations are characteristic of PAN
aneurysmal dilations
what nonspecific signs and symptoms may someone with PAN present with
fever, weight loss, malaise, weakness, h/a, abdominal pain, myalgias
what involvement is MC with PAN
RENAL involvement. . manifests as HTN, hemorrhage (in the form of microanurysms), or renal insufficiency
organ systems involved with PAN (from most to least common)
renal - htn, failure MS - arthritis, arthralgia, myalgia PNS GI Skin (raynauds too) Cardiac GU CNS
what are the diagnostic serologic tests for PAN
there are none
what labs should you get for PAN
normochromic normocytic anemia
cbc - increased leukocyte count with neutophil predom
elevated ESR
other labs that have to do with the organ involvef
how do you get the diagnosis for PAN
biopsy of involved organ (provides highest diagnostic yield)
arteriographic demonstration of involved vessels (aneurysms, stenotic segments of involved vessels)
what is the treatment for PAN
prednisone and cyclophosphamide
what does death usually result from in a person with PAN
bowel infarct, bowel perf, CV causes
what size arteries does giant cell arteritis affect?
medium and large
WHICH arteries are characteristically involved in giant cell arteritis
one or more branch of the carotid artery (particularly temporal)
also the aorta and its branches
what disease is giant cell closely associated with
polymyalgia rheumatica
what are the clinical manifestations of polymyalgia rheumatica
stiffness, aching, pain in the muscles of the neck, shoulders, lower back, hips, and thighs
increased ESR
what is the tx for PMR
low dose prednisone
what is panarteritis (in giant cell)
inflammatory mononuclear cells infiltrate the vessel wall with frequent giant cell formation and there is proliferation of the intima
how does giant cell present
fever, anemia, high ESR, headaches in a patient >50 years old
may also have malaise, weight loss, fatigue, sweats, arthraligias
may palpate a tender, thickened, rope-like temporal artery
jaw claudication
ischemis optic neuropathy (can lead to blindness)
1/3 can have lg vessel manifestations as primary presentation— SUBCLAVIAN STENOSIS - ARM CLAUDICAITION
how do you diagnose giant cell
elevated ESR (you can follow this) normochromic, normocytic anemia
clin presentation - fever, anemia, high ESR, >50 yo, +/- headache, PMR sx
diagnosis confirmed by TA biopsy
what is the pharmacotherapy for giant cell
prednisone 60mg x 1 mo.. followed by gradual tapering.
most pts req tx for >2 yrs
ASA 81 mg can reduce the occurance of cranial ischemic complications. should be added if no contraindications
what is takaysu’s arteritis
medium and large arteries, with a strong predilection for the aortic arch and its branches
what population do we see takaysu in
adolescent firls and young women
how does the path differ for giant cell versus takayasu (panarteritis)
for giant cell you have proliferation of the intima
takaysu- proliferation and fibrosis of intima and scarring of the media (thus luminal narrowing)
what is the presentation of takaysu
malaise, fever, night sweats, arthralgias, anorexia, weight loss
absent pulses of the involved vessels
HTN
lab data takayasu
elevated ESR, mild anemia
example of takaysu
young woman with decreased peripheral pulses, discrepancies in BP and arterial bruits
how is the diagnosis of takaysu confirmed
aortic arteriography or MRA (irregular vessel walls, vessel stenosis, aneurysm formation, vessel occlusion)
what is the treatment for takaysu
glucocorticoid approach
consider surgical or arterioplastic approach
