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MSK/Rheum > Vasculidities > Flashcards

Vasculidities Flashcards

1
Q

consequences of blood vessel occlusion

A

tissue ischemia

rupture of vasculature- hemorrhage

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2
Q

immune complex vasculitis

A

CTD-associated (SLE, RA)
Henoch-Schonlein purpura
cryoglobulinemia
hypersensitivity

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3
Q

ANCA-associated vasculitis

A

GPA, EGPA, MPA, renal-limited MPA

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4
Q

GPA =

A

Wegener’s granulomatosis

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5
Q

EGPA =

A

Churg-Strauss syndrome

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6
Q

MPA =

A

microscopic polyangiitis

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7
Q

Granulomatous vasculitis

A

GCA, Takajasu’s arteritis, GPA, EGPA

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8
Q

large vessel vasculitis

A

Takajasu

Giant Cell

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9
Q

medium vessel vasculitis

A

PAN
Kawasaki’s
Isolated CNS vasculitis

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10
Q

small vessel vasculitis

A 
GPA
EGPA
MPA
Henoch-Schonlein purpura
Behcet's syndrome
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11
Q

how do immune complexes generate inflammatory response

A

activate complement, generating anaphylatoxins and neutrophil chemoattractants

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12
Q

how to measure ANCA

A

indirect immunofluorescence or ELISA to PR2 and MPO

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13
Q

C-ANCA associated with

A

PR3

Wegeners granulomatosis

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14
Q

P-ANCA associated with

A

MPO

seen with Churg-Strauss, and MPA

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15
Q

ANCA titers correlate with

A

disease activity

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16
Q

ANCA binds to

A

neutrophils and monocytes, and endothelial cells

induces oxygen radical release, degranulation, inhibition of microbiocidal function, increased surface expression of PR3 on neutrophils

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17
Q

what cells are prevalent in granulomatous vasculitis

A

antigen presenting cells
T cells, cytokines
macrophages

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18
Q

Th1 cells secrete

A

IL2, IFN-gamma, TNF-alpha

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19
Q

common complaints of vasculitis

A 
fever, fatigue, weakness
rash (purpura)
arthalgias
abdominal pain
HTN, renal insufficiency
neurologic dysfunction
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20
Q

what to rule out with vasculitis ddx

A

infection
TB
malignancy

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21
Q

GCA age

A

> 50, northern european descent

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22
Q

TA age

A
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23
Q

GCA distribution

A

extracranial branches of carotid arteries

24
Q

TA distribution

A

aorta and aortic main branches

25
skip lesions associated with
GCA
26
cyclic occurrences of GCA suggst
infectious link
27
amaurosis fugax
unilateral sudden blindness due to anterior optic neurotics secondary to inflammation in posterior ciliary arteries
28
PMR is primarily linked with
GCA
29
PMR presents with
proximal shoulder and pelvic girdle pain, morning stiffness
30
treatment of GCA/PMR
corticosteroids
31
steroid sparing agents
MTX, azathioprine
32
Takajasu's arteritis is aka
pulseless disease aortic arch syndrome occlusive thromboarthropathy
33
systemic features of takayasu's arteritis
fever, malaise, weight loss, syncope, stroke, visual changes
34
diagnosis of takayasu's arteritis
angiogram, MRI angiogram, MRI, CT
35
adjunctive treatment of takayasu's arteritis
ASA, anti-platelet agents, angioplasty
36
PAN presentation
postprandial abdominal pain, numbness, foot drop
37
PAN predilection for
arterial bifurcations
38
PAN may result from
Hepatitis B infection (30%)
39
ANCA effects which size of vessels
small and medium
40
classic triad of Wegener's Granulomatosis
upper airway lower respiratory tract kidneys
41
limited WG's
relatively mild and indolent without renal involvement
42
laboratory features of GPA
C-ANCA (PR3 >>MPO) | anemia, thrombocytosis, elevated ESR/CRP
43
pathologic features of GPA
granulomas, vasculitis, necrosis
44
most common presenting feature of GPA
upper airway disease (70% of patients at onset)
45
other common presentations of GPA
otologic manifestations- serious otitis media nasal disease
46
treatment of GPA
cyclophosphamide oral and prednisone- initial treatment
47
prodrome of EGPA
dominated by allergic features. Allergic rhinitis and asthma, atopy
48
tissue eosinophilia present in
Churg-Strauss syndrome
49
EGPA treatment
corticosteroids | cyclophosphamide for severe/refractory disease
50
glomerulonephritis common in
microscopic polyangiitis
51
Behcet's disease triad
oral ulcers, genital ulcers, andterior uveitis
52
Behcet's disease is associated with
HLA-B51
53
Henoch Schonlein Purpura aka
anaphylactoid purpura
54
HSP tetrad
palpable purpura arthralgias GI involvement glomerulonephritis
55
main treatments of vasculidities
``` corticosteroids cyclophosphamide MTX azathioprine cyclosporine MMF plasmapharesis IVGG trimethoprim-sulfamethoxazole biologics (rituximab) ```

MSK/Rheum (5 decks)

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