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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
Q

skip lesions associated with

A

GCA

26
Q

cyclic occurrences of GCA suggst

A

infectious link

27
Q

amaurosis fugax

A

unilateral sudden blindness due to anterior optic neurotics secondary to inflammation in posterior ciliary arteries

28
Q

PMR is primarily linked with

A

GCA

29
Q

PMR presents with

A

proximal shoulder and pelvic girdle pain, morning stiffness

30
Q

treatment of GCA/PMR

A

corticosteroids

31
Q

steroid sparing agents

A

MTX, azathioprine

32
Q

Takajasu’s arteritis is aka

A

pulseless disease
aortic arch syndrome
occlusive thromboarthropathy

33
Q

systemic features of takayasu’s arteritis

A

fever, malaise, weight loss, syncope, stroke, visual changes

34
Q

diagnosis of takayasu’s arteritis

A

angiogram, MRI angiogram, MRI, CT

35
Q

adjunctive treatment of takayasu’s arteritis

A

ASA, anti-platelet agents, angioplasty

36
Q

PAN presentation

A

postprandial abdominal pain, numbness, foot drop

37
Q

PAN predilection for

A

arterial bifurcations

38
Q

PAN may result from

A

Hepatitis B infection (30%)

39
Q

ANCA effects which size of vessels

A

small and medium

40
Q

classic triad of Wegener’s Granulomatosis

A

upper airway
lower respiratory tract
kidneys

41
Q

limited WG’s

A

relatively mild and indolent without renal involvement

42
Q

laboratory features of GPA

A

C-ANCA (PR3&raquo_space;MPO)

anemia, thrombocytosis, elevated ESR/CRP

43
Q

pathologic features of GPA

A

granulomas, vasculitis, necrosis

44
Q

most common presenting feature of GPA

A

upper airway disease (70% of patients at onset)

45
Q

other common presentations of GPA

A

otologic manifestations- serious otitis media

nasal disease

46
Q

treatment of GPA

A

cyclophosphamide oral and prednisone- initial treatment

47
Q

prodrome of EGPA

A

dominated by allergic features. Allergic rhinitis and asthma, atopy

48
Q

tissue eosinophilia present in

A

Churg-Strauss syndrome

49
Q

EGPA treatment

A

corticosteroids

cyclophosphamide for severe/refractory disease

50
Q

glomerulonephritis common in

A

microscopic polyangiitis

51
Q

Behcet’s disease triad

A

oral ulcers, genital ulcers, andterior uveitis

52
Q

Behcet’s disease is associated with

A

HLA-B51

53
Q

Henoch Schonlein Purpura aka

A

anaphylactoid purpura

54
Q

HSP tetrad

A

palpable purpura
arthralgias
GI involvement
glomerulonephritis

55
Q

main treatments of vasculidities

A 
corticosteroids
cyclophosphamide
MTX
azathioprine
cyclosporine
MMF
plasmapharesis
IVGG
trimethoprim-sulfamethoxazole
biologics (rituximab)

MSK/Rheum (5 decks)

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