Term
|
Definition
| inflammation of and damage to walls of blood vessels |
|
|
Term
| what vessels does vasculitis affect, what vessels are in each category |
|
Definition
large (elastic): aorta, aortic brnaches, pulmonary arteries
medium (muscular): small branches of aorta (coronary and renal A)
small arteries and arterioles |
|
|
Term
| what are 5 common infections that cause vasculitis |
|
Definition
bacterial: neisseria
rickettsial: RMSF
spirochetal: syphilis
fungal: aspergullosis
viral: HSV |
|
|
Term
| what are 3 ways the immune system causes vasculitis |
|
Definition
immune complexes
antineutrophil cytoplasmic antibody (ANCA) mediated
direct antibody mediated |
|
|
Term
| what are 3 diseases that use immune complexes to causes vasculitis |
|
Definition
henoch schonlein purpura
SLE
RA |
|
|
Term
| what are 3 diseases that use ANCA to cause vasculitis |
|
Definition
wegener granulomatosis
microscopic polyangiitis
churg strauss syndrome |
|
|
Term
| what are two diseases that use antibodies to cause vasculitis, what are the names of the antibodies |
|
Definition
kawasaki disease: anti-endothelial
goodpasture syndrome: anti-glomerular basement membrane |
|
|
Term
| what are three diseases that use the immune system to cause vasculitis but we dont know how they do it |
|
Definition
giant cell (temporal arteritis)
takaysu arteries
polyarteritis nodosa |
|
|
Term
| what are 4 general signs in someone who has vasculitis |
|
Definition
| fever, malaise, arthragias, weight loss |
|
|
Term
| what 2 vasculitis syndromes like large vessels |
|
Definition
temporal arteritis
takayasu |
|
|
Term
| what 2 vasculitis syndromes like medium vessels |
|
Definition
|
|
Term
| what vasculitis syndromes like small vessels |
|
Definition
everything else
ANCA associated vasculitis |
|
|
Term
|
Definition
anti-neutrophil cytoplasmic antibodu
antibodies to proteins inside neutrophil. body becomes sensitized to them because there is something that causes the protein to be expressed and move to the surface of cells |
|
|
Term
| what things can cause proteins that ANCA attacks to move to the cell surface |
|
Definition
|
|
Term
| what are the two types of ANCA, what do they attack |
|
Definition
perinuclear (p): antibodies to myeloperoxidase
cytoplasmic (C): antibodies to protenase (PR) 3 in the cytoplasm |
|
|
Term
| what can the presence of ANCA diagnose |
|
Definition
it cant.
it only helps diagnose.
negative does not RO disease
positive does not RI disease |
|
|
Term
| what are commonalities in people who develop ANCA (3) |
|
Definition
respiratory tract infection
older
caucasian |
|
|
Term
| what three vasculitis are due to ANCA, which ANCA |
|
Definition
wegerners- cytoplasmic
microscopic polyangiitis- perinuclear
churg struss syndrome - perinuclear |
|
|
Term
| clinical abnormalities that suggest vasculitis (7) |
|
Definition
palpable purpura
pulmonary infiltrates
microscopic hematuria
chronic inflammatory sinusitis
mononeuritis multiplex
unexplained ischemic events
glomerulonephritis with evidence of multisystem disease |
|
|
Term
| why is it essential you RO infection as a cause to vasculitis |
|
Definition
| immunosupressive treatment is often used |
|
|
Term
| what are infectious conditions that can mimic vasculitis (8) |
|
Definition
bacterial endocarditis
disseminated gonoccal infection
pulmonary histoplasmosis
coccidioidomycosis
syphilis
lyme disease
RMSF
whipple's disease |
|
|
Term
| when do you angiogram or biopsy for vasculitis |
|
Definition
| when you have STRONG evidence |
|
|
Term
| what is the most common form of vasculitis |
|
Definition
| giant cell (temporal) arteritis |
|
|
Term
| who gets giant cell arteritis |
|
Definition
female > male
elderly, rare <50yo |
|
|
Term
| where is giant cell arteritis (6) |
|
Definition
small and medium arteries
cranial arteries: temporal (headache), facial, opthalamic (blindness)
aortic arch (rare) |
|
|
Term
| what are the early symptoms of giant cell arteritis (3) |
|
Definition
| fever, malaise, weight loss |
|
|
Term
| what are the symptoms of temporal arterieis (6) |
|
Definition
weak pulse in UE
occular: blurred vision and blindness due to opthalamic A
systemic HTN due to renal A narrowing
throbbing headache (temporal A)
artery is very tender |
|
|
Term
| what are the temporal arteritis lab findings (1) |
|
Definition
| elevated ESR due to immune mediated inflammation - very non-specific |
|
|
Term
| what do the arteries look like in temporal arteritis (4) |
|
Definition
nodular thickening
granulomatous inflammation
fragmentation of internal elastic lamina (due to inflammation)
T cell dependent disease |
|
|
Term
| how is temporal arteritis diagnosed (1) |
|
Definition
biopsy of temporal artery
classic presentation |
|
|
Term
| how is temporal artritis treated |
|
Definition
|
|
Term
| what is the prognosis of temporal arteritis |
|
Definition
treated: excellent
untreated: blindness |
|
|
Term
| which condition is very similar to takayasu arteritis |
|
Definition
| giant cell/temporal arteritis |
|
|
Term
| who gets takaysau arteritis (3) |
|
Definition
|
|
Term
| where is takaysau arteritis (4) |
|
Definition
large to medium sized arteries
aortic arch
major aortic branches |
|
|
Term
| what are early signs of takaysau arteritis (3) |
|
Definition
| fever, weight loss, malaise |
|
|
Term
| what are signs of takaysau arteritis (6) |
|
Definition
loss of pulse in UE
ocular disturbances
retinal hemorrhage
neurological abnormalities
HTN from renal A stenosis
stroke from carotid A occlusion |
|
|
Term
| what does the artery look like in takaysau arteritis takaysau arteritis (2) |
|
Definition
granulomatous vasculitis
fibrous thickening of artery wall |
|
|
Term
| how is takaysau arteritis diagnosed (2) |
|
Definition
|
|
Term
| what is the treatment for takaysau arteritis (2) |
|
Definition
prednisone for symptoms
surgical correction of stenosed vessels |
|
|
Term
| what is the prognosis of takaysau arteritis (2) |
|
Definition
variable
chronic and relapsing |
|
|
Term
| who gets polyarteritis nodosa (PAN) (3) |
|
Definition
young adults
male > female
30% have HepB antigen |
|
|
Term
| where is polyarteritis nodosa (PAN) (3) |
|
Definition
small to medium arteries
SYSTEMIC vasculitis EXCEPT pulmonary capillaries or glomeruli |
|
|
Term
| what arteries is polyarteritis nodosa (PAN) most frequently in (most to least (9)) |
|
Definition
kidney
heart
liver
GI
pancreas
testis
skeletal muscle
nervous system
skin |
|
|
Term
| what are the common initial symptoms of polyarteritis nodosa (PAN) (2) |
|
Definition
sudden elevation in BP due to kidney involvement
abdominal pain and melena (blood in stool) |
|
|
Term
| what are the symptoms of polyarteritis nodosa (PAN) (8) |
|
Definition
fever
malaise
weight loss
hematuria
renal failure
HTN: rapid onsetmuscular aches and pains
peripherial neuritis (mononeuritis)
livedo reticularis |
|
|
Term
| what is livedo reticularis |
|
Definition
| skin lesion in dependent areas in polyarteritis nodosa (PAN) |
|
|
Term
| what are the lab findings in polyarteritis nodosa (PAN) (4) |
|
Definition
disturbances in BUN and CR
elevated ESR
low complement |
|
|
Term
| what does the artery look like in polyarteritis nodosa (PAN) (4) |
|
Definition
segmental necrotizing vasculitis
thrombosis
infarction
aneurysms |
|
|
Term
| how is polyarteritis nodosa (PAN) diagnosed (2) |
|
Definition
arterial biopsy (hopefully from livedo reticularis skin lesion)
angiography |
|
|
Term
| how is polyarteritis nodosa (PAN) treated (2) |
|
Definition
corticosteroids: decrease inflammation
cyclophosphamide |
|
|
Term
| how does cyclophosphamide work |
|
Definition
| chemotherapeutic drug that targets cells replicating rapidly like marrow which knocks down immune system helping in vasculitis |
|
|
Term
| what is the prognosis of polyarteritis nodosa (PAN) |
|
Definition
treated 90% long term remission
untreated is fatal |
|
|
Term
| who gets kawasaki disease (4) |
|
Definition
kids <4yo
japan, hawaii, main US |
|
|
Term
| where is kawasaki disease (4) |
|
Definition
large, medium, small arteries
70% in coronary artery
antibody attacks endothelium which is in ALL vessels |
|
|
Term
| signs of kawasaki disease (9) |
|
Definition
fever
lymphadenopathy
conjunctivitis: bilateral, no exudate
erythema/cracking of lips
strawberry tongue
erythema and erosions of oral mucosa
edema of hands and feet
desqumation of fingertips
polmorphous exanthema |
|
|
Term
| what are the lab findings in kawasaki disease (2) |
|
Definition
|
|
Term
| what do the vessels look like in kawasaki disease (3) |
|
Definition
necrotizing vasculitis
anti-endothelial antibodies
aneurysms |
|
|
Term
| how is kawasaki disease officially diagnosed (6) |
|
Definition
fever for 5d+ and 4 of the following...
1. acute changes in extremities: erythema/edema of hands/feet
2. convalescent changes in extremities: membranous desquamation of fingertips
3. polmorphous exanthema: rash with many sizes and shapes
4. bilateral conjunctival infection without exudates (blood shot eyes)
5. erythema and cracking of lips/strawberry tongue
cervical lymphadenopathy >1.5cm |
|
|
Term
| treatment of kawasaki disease (2) |
|
Definition
aspirin
IV immunoglobulins |
|
|
Term
| prognosis of kawasaki disease (3) |
|
Definition
treated: coronary artery disease risk 4%
untreated: coronary artery disease risk 20%
may resolve spontaneously |
|
|
Term
| who gets microscopic polyangiitis (MPA) (2) |
|
Definition
40-60yo avg 57yo
male > females |
|
|
Term
| where is microscopic polyangiitis (MPA) |
|
Definition
small, medium, capillaries, venules
skin, mucous membranes, lungs, brain, heart, GI tract, kidneys, muscle |
|
|
Term
| what are the signs of microscopic polyangiitis (MPA) (12) |
|
Definition
fever
weight loss
myalgias
acute
GLOMERULONEPHRITIS common
hemoptysis
hematuria
proteinuria
GI pain or bleeding
palpable purpura
mononeuritis: can progress to involve multiple sites |
|
|
Term
| how is microscopic polyangiitis (MPA) diagnosed (4) |
|
Definition
p-ANCA in 75%
elevated ESR
leukocytosis
thrombocytosis |
|
|
Term
| what do the arteries look like in microscopic polyangiitis (MPA) (3) |
|
Definition
fibrnoid necrosis of media
leykocytoclastic angiitis
RBC casts in urine: their close and stick thgother in damaged glomeruli |
|
|
Term
| how is microscopic polyangiitis (MPA) diagnosed (1) |
|
Definition
|
|
Term
| how is microscopic polyangiitis (MPA) treated (1) |
|
Definition
| remove inciting agents: drug antigens, microorganisms, tumors |
|
|
Term
| what is the prognosis of microscopic polyangiitis (MPA) (4) |
|
Definition
treated: 75% 5y survival
prednisone
daily cyclophosphamide if life threatning
35% have disease relapse |
|
|
Term
| what needs to be excluded when diagnosing kawasaki disease (9) |
|
Definition
staph infection: SSSS, TSS
strap infection: scarlet fever
measles, other viral exanthems
leptospirosis
rickettsial disease
SJS, drug reaction
juvenile RA |
|
|
Term
| who gets henoch-schonlein pupura (2) |
|
Definition
usually kids 4-7yo
usually follows respiratory infection |
|
|
Term
| where is henoch-schonlein pupura |
|
Definition
|
|
Term
| what are the signs of henoch-schonlein pupura (8) |
|
Definition
palpable purpura (butt and lower extremities)
arthralgias
GI: colicky abdominal pain, nausea, vomiting, diarrhea, constipation, blood mucous in stool
glomerulonephritis- rare |
|
|
Term
| what are the lab signs of henoch-schonlein pupura (3) |
|
Definition
mild leukocytosis
necrotizing vasculitis
IgA deposition in lesions detectable by flourescence microscopy |
|
|
Term
| how is henoch-schonlein pupura officially diagnosed (3) |
|
Definition
rash
GI complaints or hematuria
arthritis |
|
|
Term
| henoch-schonlein pupura treatmetn |
|
Definition
| prednisone alleviates symptoms |
|
|
Term
| henoch-schonlein pupura prognosis |
|
Definition
mortality rare (1-5% due to end stage renal disease)
10-40% disease reocurrance |
|
|
Term
| who gets wegner granulomatosis (2) |
|
Definition
|
|
Term
| where is wegner granulomatosis (5) |
|
Definition
small and medium arteries
lower lung and upper respiratory
kidneys |
|
|
Term
| what are the signs of wegner granulomatosis (4) |
|
Definition
bilateral pneumonia
chronic sinusitis
nasopharyngeal ulcerations
evidence of renal disease |
|
|
Term
| what are the lab findings in wegner granulomatosis |
|
Definition
|
|
Term
| what do the vessels look like in wegner granulomatosis (3) |
|
Definition
acute necrotizing granulomas of upper respiratory tract
necrotizing granulomas vaculitis
renal disease: crescentic glomerulonephritis |
|
|
Term
| how is wegner granulomatosis diagnosed |
|
Definition
| arterial biopsy: evidence of necrotizing granulomatous lesions in vessels |
|
|
Term
| how is wegner granulomatosis treated (2) |
|
Definition
cyclophosphamide
corticosteroids |
|
|
Term
| what is the prognosis of wegner granulomatosis |
|
Definition
treated: 99% long term remission
untreated: 80% 1 year mortality |
|
|
Term
| who gets buerger disease (thromboangiitis obliterans)(7) |
|
Definition
usuallu <35 yo
male > female
heavy cigarette smoke damages vessels
most common in isreal, india, japan, south america |
|
|
Term
| where is buerger disease (thromboangiitis obliterans)(5) |
|
Definition
small and medium arteries
tibial and radial arteries
extends into veins and nerves |
|
|
Term
| signs of buerger disease (thromboangiitis obliterans)(4) |
|
Definition
superificial nodular phlebitis
raynaud phenomenon
instep claudication
chronic ulceration of extremities (gangrene) |
|
|
Term
| what are the lab findings of buerger disease (thromboangiitis obliterans)(1) |
|
Definition
| negative for acute phase reactants |
|
|
Term
| what do the vessels look like in buerger disease (thromboangiitis obliterans)(4) |
|
Definition
granulomatous inflammation
thrombosos if lumen
microabscesses
arteries, veins, nerves encased by fibrous tissue |
|
|
Term
| how is buerger disease (thromboangiitis obliterans)diagnosed (2) |
|
Definition
exclude other diseases
angiography |
|
|
Term
| how is buerger disease (thromboangiitis obliterans)treated (1) |
|
Definition
|
|
Term
| what is the prognosis of buerger disease (thromboangiitis obliterans) |
|
Definition
|
|
Term
| who gets chrug-strauss syndrome (3) |
|
Definition
any age usually 15-70yo, avg 48yo
vasculitis develops within 3y of asthma onset |
|
|
Term
| where is chrug-strauss syndrome (8) |
|
Definition
small and medium arteries
lung involvement predominates
skin, CV, kidney, PNS, GI tract |
|
|
Term
| signs of chrug-strauss syndrome (6) |
|
Definition
fever
weight loss
malaise
severe asthmatic attacks
pulmonary infiltrates
72% exhibit mononeuritis multiplex
51% have skin lesions (purpura, nodules) |
|
|
Term
| what are the lab findings for chrug-strauss syndrome (3) |
|
Definition
eosinophilis >1000 cells/ul or >10%
elevated ESR
p-ANCA in 48% |
|
|
Term
| what do the vessels look like in chrug-strauss syndrome (2) |
|
Definition
granulomatous inflammation
infiltration of tissues with eosinophils |
|
|
Term
| how is chrug-strauss syndrome diagnosed (4) |
|
Definition
biopsy
asthma
peripherial eosinophilia
clinical features |
|
|
Term
| how is chrug-strauss syndrome treated |
|
Definition
|
|
Term
| what is the prognosis of chrug-strauss syndrome (3) |
|
Definition
treated: 75% 5y survival
untreated: 25% 5y survival
mycardial involvement most frequent cause of death |
|
|
Term
| what are the types of cryoglobulinemia (2) |
|
Definition
type 1: lymphoproliferative diseases, chronic infections
type II: drug users (usually with hep C infection) |
|
|
Term
| where is cryoglobulinemia (2) |
|
Definition
|
|
Term
| what are the signs of cryoglobulinemia 1 (1) |
|
Definition
|
|
Term
| what are the signs of cryoblobulinemia 2 (6) |
|
Definition
mild recurrent bouts of LE purpura
glomerulonephritis, arthralgia, distal necrosis, abd pain, peripherial neuropathy |
|
|
Term
| what are the lab signs of cryoglobulinemia (4) |
|
Definition
cryoglobulins: percipitate at cold temp so lesions show in hands and feet
rheumatoid factor
hypocompletenemia (esp C4)
usually hepC positive |
|
|
Term
| what do the vessels look like in cryoglobulinemia (2) |
|
Definition
leukocytoclastic vasculitis
proliferative glomerulonephritis with IgM capillary loops |
|
|
Term
| how is cryoglobulinemia diagnosed (7) |
|
Definition
cutaneous vasculitis
hypocomplemtemeia
elevated liver enzymes
hepatomeagly
caput medusa
cirosis
cryoglobulinemia |
|
|
Term
| treatment of cryoglobulinemia (3) |
|
Definition
mild to moderate: interferon a if hepC
severe: immunosupressive therapy (steroids) and or plasmapheresis |
|
|
Term
| who gets bechet's disease (3) |
|
Definition
old silk route japan to mediterranean
mid 20-30s
genetic predisposition HLA-B51 |
|
|
Term
|
Definition
|
|
Term
| signs of bechet's disease (9) |
|
Definition
A/P uveitis, can cause blindness
aphthous ulcers in mouth
headaches, confusion, stroke due to white matter involvement and aseptic meninges
folliculitis
pulmonary artery aneurysm
arthralgias
GI ulcers- mouth and internall
genital ulcers |
|
|
Term
| lab findings for bechet's disease (1) |
|
Definition
| postive pathergy test is SUPPORTIVE: blank sterile needle makes prick and mark it, several days leter the site has palpable reaction to skin |
|
|
Term
| what do vessels look in in bechet's disease (2) |
|
Definition
neutrophilc vascular reaction
+/- fibrinoid necrosis |
|
|
Term
| how is bechet's disease diagnosed (6) |
|
Definition
biopsy if possible
supportive findings
recurrent for 3y
oral ulcerations
uveitis
skin lesions |
|
|
Term
| how is bechet's treated (3) |
|
Definition
topical steroids if mucocutaneous
systemic corticosteroids or immunosupressents |
|
|
Term
| what is the prognosis of bechet's (3) |
|
Definition
most relapse and remitt
poor if CNS involved
most common morbidity: blindness |
|
|
Term
| cause of cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) |
|
Definition
7-21d after exposure to antigen (iusually drug, less commonly vaccination)
circulating immune complexes form after drug exposure |
|
|
Term
| what drugs trigger cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) (7) |
|
Definition
anti-HTN
anti-convulsants
anti-thyroid
GF
antibiotics
NSAIDS
SSRI |
|
|
Term
| where is cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) |
|
Definition
|
|
Term
| signs of cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) (3) |
|
Definition
palpable purpure usually in LE and dependent areas
confined to skin
1-3 wks after starting a drug |
|
|
Term
| what do vessels look like in cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) |
|
Definition
| isolated cutaneous leukocytoclastic angiitis |
|
|
Term
| how is cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) diagnosed |
|
Definition
| skin lesions with hx of exposure to new agent |
|
|
Term
| how is cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) treated (2) |
|
Definition
withdrawl of offending agent
corticosteroids if needed |
|
|
Term
| what is the prognosis of cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) (2) |
|
Definition
complete recovery common
skin hyperpigmentation may me sequela |
|
|
