Icm Rheum 06: Vasculitides

Front 1

4 steps in vasculitis pathophysiology

Back 1

vessel inflammation
vessel necrosis
vessel obstruction
tissue ischemia

Front 2

5 skin lesions characteristic of vasculitis

Back 2

non-blanching palpable purpura
splinter hemorrhage
periungeal infarcts
urticaria which bleeds or lasts >24h
nodose lesions

Front 3

6 kinds of vasculitis

Back 3

leukocytoclastic/hypersensitivity
CT disorders
polyarteritis group
granulomatous
giant cell arteritis
miscellaneous

Front 4

leukocytoclastic vasculitis affects (2)

Back 4

capillaries
venules

Front 5

CT diseases affect (2)

Back 5

arterioles
venules

Front 6

polyarteritis group affects

Back 6

small/medium muscular arteries

Front 7

granulomatous group affects

Back 7

small/medium vessels

Front 8

giant cell arteritis affects

Back 8

large vessels

Front 9

3 vasculitis groups

Back 9

large vessel
medium vessel
small vessel

Front 10

3 large vessel vasculitides

Back 10

giant cell arteritis
Takayasu's arteritis
primary granulomatous CNS vasculitis

Front 11

2 medium vessel vasculitides

Back 11

polyarteritis nodosa
Kawasaki's disease

Front 12

2 kinds of small vessel vasculitides

Back 12

pauci-immune
immune complex

Front 13

3 pauci-immune small vessel vasculitides

Back 13

microscopic polyangiitis
Wegener's
Churg-Strauss

Front 14

4 mechanisms for vasculitis

Back 14

granulomas (cell mediated)
anti-neutrophil Abs
anti-endothelium Abs
immune complexes

Front 15

___ is a model for IC vasculitis

Back 15

Arthus reaction

Front 16

all ___ vasculitides are granulomatous
other granulomatous vasculitides are ___ (3)

Back 16

large artery
Wegener's
Churg-Strauss
Kawasaki

Front 17

hypersensitivity vascullitis is aka

Back 17

cutaneous leukocytoclastic vasculitis (CLV)

Front 18

CLV is often limited to ___

Back 18

skin

Front 19

CLV is a ___ disease.
complement is low/normal/high
ESR is low/normal/high

Back 19

IC
low
high

Front 20

CLV usually occurs ____ after exposure to allergen

Back 20

7-10 days

Front 21

CLV occasionally affects ___ but rarely ___ or ___.

Back 21

kidneys
heart
CNS

Front 22

when diagnosing CLV it is important to exclude ___, especially ___ (2)

Back 22

infection
HBV
HCV

Front 23

most CLV patients respond to ___
25% require ___. 15% require ___.

Back 23

discontinuation of causing agent
NSAIDs
CS

Front 24

T/F: PAN usually affects lungs

Back 24

false: lungs usually spared

Front 25

PAN is more common in men/women

Back 25

men (1.5:1)

Front 26

PAN is associated with ___ (2)

Back 26

HBV
hairy cell leukemia

Front 27

inflammation in PAN is ___. 2 common results of the fibrinoid necrosis are ___

Back 27

segmental
vessel occlusion
aneurysm

Front 28

3 systemic symptoms in PAN

Back 28

weakness
arthralgia
fever

Front 29

4 systemic signs of PAN

Back 29

HTN
renal failure
neuro dysfunction

Front 30

weight loss criterion for PAN

Back 30

>= 4kg

Front 31

skin criterion for PAN

Back 31

livedo reticularis

Front 32

pain in ___ is a criterion for PAN
also, ___ not in ___

Back 32

testicle
diffuse myalgia
shoulder girdle

Front 33

neuro criterion for PAN

Back 33

mononeuropathy or polyneuropathy

Front 34

BP criterion for PAN

Back 34

diastolic > 90 mm Hg

Front 35

2 renal criteria for PAN

Back 35

BUN > 40 mg%
creatinine > 1.5 mg%

Front 36

infectious criterion for PAN

Back 36

HBV

Front 37

radiological criterion for PAN

Back 37

arteriographic abnormality: aneurysm or occlusion

Front 38

biopsy criterion for PAN

Back 38

granulocytes

Front 39

you have PAN if you have at least ___ of the criteria

Back 39

3

Front 40

renal syndrome associated with PAN

Back 40

FPGN

Front 41

5 year suvival of PAN with treatment is ___. main causes of death are ___ (3)

Back 41

80%
renal failure
GI infarction
MI
CVA

Front 42

recurrences of PAN are more/less frequent than in Wegener's

Back 42

less

Front 43

50% of PAN patients get remission with ___ alone. other half needs ___ also.

Back 43

CS
cyclophosphamide

Front 44

anti TNF works well for ___ but less well for ___.

Back 44

RA
PAN

Front 45

classic wegener's affects ___ (2)
limited form of wegeners only affects ___. both may also affect ___.

Back 45

RT (upper and lower)
kidneys
RT
other organs

Front 46

____% of Wegener's is ANCA positive. ___% of limited Wegener's is ANCA positive.

Back 46

90
55-60

Front 47

renal syndrome commonly caused by Wegener's.

Back 47

RPGN

Front 48

Wegener's commonly presents with 1 or more of these 4 kinds of problems

Back 48

nasal
polyarthralgia
myalgia
lower RT

Front 49

4 nasal problems in Wegener's

Back 49

persistent rhinorrhea
purulent/bloody nasal discharge
nasal/oral ulcers
sinus pain

Front 50

6 lower RT problems in Wegener's

Back 50

cough
dyspnea
hemoptysis
pleuritic pain
pleural effusion
consolidation

Front 51

___ complements IFA. it has higher ___ (2)

Back 51

ELISA
specificity
PPV
(didn't we learn otherwise in immuno?)

Front 52

P-ANCA is associated with ____ in ELISA

Back 52

anti-MPO

Front 53

C-ANCA is associated with ___ in ELISA

Back 53

anti-PR3

Front 54

Wegener's is associated with ___-ANCA

Back 54

C

Front 55

in ANCA-mediated diseases, an attack on ___ causes release of ___ which causes ___

Back 55

neutrophils
inflammatory cytokines
attack on endothelial cells

Front 56

4 classification criteria for Wegener's
you need ___ of them for a dx

Back 56

oral/nasal inflammation
abnormal CXR
urinary sediment
granulomas in vessel biopsy
2

Front 57

facial deformity associated with Wegener's

Back 57

saddle nose

Front 58

saddle nose is caused by ___

Back 58

destruction of nasal septum

Front 59

CXR abnormality associated with Wegener's

Back 59

nodules

Front 60

mortality in untreated Wegener's pts. within 2 years

Back 60

90%

Front 61

gold standard treatment for Wegeners is ___ (2)

Back 61

cyclophosphamide (CTX)
CS

Front 62

in mild disease, ____ is preferred to CTX because ____

Back 62

methotrexate (MTX)
it's safer

Front 63

prophylaxis against ___ is required for Wegener's patients, because ___.
use ___ for this, but not if you're using ___ against the Wegener's, because ___.

Back 63

PCP
they're immunosuppressed
cotrimoxazole
MTX
cotrimoxazole increases MTX bioavailability

Front 64

maintenance therapy for WG lasts ___. it includes ____ (3) but doesn't include ___

Back 64

18 months
azathioprine
methotrexate
cotrimoxazole
GCs

Front 65

infliximab is a ___.
etanercept is a ___.
___ is more effective in WG

Back 65

anti-TNF mAb
synthetic soluble TNFR
infliximab

Front 66

giant cell arteritis almost never occurs before age ___. it is most common in ___ (ethnicity)

Back 66

50
scandinavians

Front 67

GCA mostly affects ___ arteries

Back 67

aortic arch branches

Front 68

the most serious effect of GCA is ___

Back 68

vision loss

Front 69

___ is a very specific complaint for GCA
other complaints include (5)

Back 69

jaw claudication
new headache in temporal region
visual symptoms
polymyalgia rheumatica
arthritis
arm claudication

Front 70

claudication is ___

Back 70

pain from ischemia

Front 71

2 late complications of GCA

Back 71

aneurysims
aortic dissection

Front 72

2 cardio findings in GCA

Back 72

decreased pulse
BP difference between arms

Front 73

2 GCA arthropathies

Back 73

limited motion at shoulder, hips
synovitis

Front 74

2 lab findings in GCA

Back 74

ESR
CRP

Front 75

___ is very high in GCA and may reflect ___.

Back 75

IL-6
disease activity

Front 76

5 diagnostic criteria for GCA.
you need ___ to diagnose

Back 76

age > 50
new headache
temporal artery abnormality
high ESR
granulomas on vessel biopsy
3

Front 77

T/F: biopsy has high sensitivity for GCA

Back 77

false

Front 78

treatment for GCA is with ___. initial dose is ___ and then tapers to ___

Back 78

GCs
40-60 mg
10 mg

Front 79

adjunct therapy for GCA includes ___ (2)

Back 79

MTX
infliximab

Front 80

takayasu's arteritis affects ___ sized vessels. ____% of cases are in men.
age of onset is ___

Back 80

large
10-20
30-40

Front 81

vessel most commonly affected in takayasu is ___. the histology of the lesions resembles ___.

Back 81

proximal aorta
GCA

Front 82

T/F: CNS symptoms may be present in takayasu's.

Back 82

true

Front 83

visual impairment in takayasu's is due to ___

Back 83

cerebral ischemia

Front 84

2 peripheral symptoms of takayasu's

Back 84

cool extremities
arm/leg claudication

Front 85

3 cardiac symptoms of takayasu's

Back 85

angina pectoris
MI
aortic regurg

Front 86

hypertension is present in ___% of takayasu's and is due to ___

Back 86

50
renal artery stenosis

Front 87

surgical intervention in takayasu's is rare/common

Back 87

common

Front 88

T/F: takayasu's is a self-limiting disease

Back 88

false: chronic

Front 89

on angiography, luminal narrowing in Takayasu's is sharp/gradual

Back 89

gradual

Front 90

6 diagnostic criteria for takayasu's
___ required for dx

Back 90

age <40
claudication of extremities
decreased brachial artery pulse
BP difference >10 mm Hg
subclavian/aortic bruits
abnormal arteriogram
3

Front 91

___ is main treatment for takayasu's.

Back 91

GC

Front 92

Churg-Strauss is a variant of ___ and is characterized by persistent ___ and mild ___

Back 92

eosinophilia
renal symptoms