Vascular Pathology: Vasculitis Flashcards

1
Q

what are three layers of the arterial wall

A

endothelial intima
smooth muscle media
connective tissue adventitia

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2
Q

What is the etiology of vasculitis

A

unknown

not infectious

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3
Q

what are 2 broad categories of clinical symptoms for vasulitis

A
  1. nonspecific symptoms of inflammation (fever, fatigue, weight loss, myalgias)
  2. symptoms of organ ischemia
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4
Q

what can cause symptoms of organ ischemia

A
  1. luminal narrowing

2. thrombosis of inflamed vessels

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5
Q

large-vessel vasculitis involves what

A

aorta and its major branches

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6
Q

medium-vessel vasculitis involves

A

muscular arteries that supply organs

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7
Q

small-vessel vasculitis involves

A

arterioles, capillaries and venules

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8
Q

Name 2 large-vessel vasculitis

A
  1. Temporal (Giant Cell) Arteritis

2. Takayasu Arteritis

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9
Q

Temporal ( Giant Cell ) Arteritis is commonly found in who

A

older adults greater than 50

female

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10
Q

clinical symptom for temporal ( giant cell) arteritis and where the vasculitis has to occur for that symptom

A

headache ( temporal artery)
visual disturbances (ophthalmic artery)
jaw claudication
flu-like with joint and muscle pain (polymyalgia rheumatica)

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11
Q

What would be abnormal lab values for temporal ( giant cell) arteritis

A

elevated ESR greater than 100

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12
Q

what do ESR values mean?

A

erythrocyte sedimentation rate
red blood cells sediment in a period of one hour
hematology test
non-specific measure of inflammation

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13
Q

what will the biopsy show for temporal (giant cell )arteritis

A

inflamed vessel wall with giant cells and intimal fibrosis
segmental
-must do biopsy of long segmnet of vessel

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14
Q

how is temporal (giant cell) arteritis treated

A

corticosteroids

  • need immediate treatment
  • high risk of blindness without treatment
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15
Q

Where does takayasu arteritis occur

A

aortic arch at branch points

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16
Q

Takayasu arteritis occurs in who

A

adults less than 50

young asian females

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17
Q

clinical symptoms of takayasu arteritis

A

visual and neurological symptoms with weak or absent pulse in upper extremity (Pulseless disease)

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18
Q

what are abnormal lab values for Takayasu

A

ESR elevated

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19
Q

treatment for Takayasu

A

corticosteroids

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20
Q

what is the difference between Temporal (giant cell) arteritis and Takayasu

A

Temporal: branches of carotid artery
Takayasu: aortic arch at branch points

temporal: greater 50 years
Takayasu: less 50 years, asian

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21
Q

Name 3 medium-vessel vasculitis

A
  1. polyarteritis nodosa
  2. Kawasaki disease
  3. Buerger disease
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22
Q

Polyarteritis Nodosa is what

A
  • Necrotizing vasculitis involving multiple organs

- spares lungs

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23
Q

Polyarteritis nodosa commonly presents in who

A

young adults

24
Q

what are clinical symptoms for polyarteritis nodosa

A

hypertension ( renal artery )
abdominal pain with melena ( mesenteric artery)
neurological disturbances
skin lesions

25
melena
dark sticky feces containing partly digested blood
26
what are abnormal values associated with polyarteritis nodosa
associated with serum HBsAg | hepatitis B surface antigen
27
How do lesions present in polyarteritis nodosa
- varying stages present at once - Early: transmural inflammation with fibrinoid necrosis - early then heals making 'string-of-pearls' appearance
28
treatment for polyarteritis nodosa
corticosteroids and cyclophosphamide | - fatal if not treated
29
Kawasaki disease classically affects what group of people
asian children less than 4 yrs old
30
how does Kawasaki present
non-specific signs: fever, conjunctivitis, erythematous rash of palms and soles, enlarged cervical lymph node
31
what part of the body is commonly involved with Kawasaki disease? what does this lead to
coronary artery 1. thrombosis with myocardial infarction 2. aneurysm with rupture
32
what is the treatment for Kawasaki
aspirin and IVIG | disease is self-limited
33
what is the caution with giving aspirin with an asian child presenting with Kawasaki
clinical features of Kawasaki similar to viral disease - not suppose to give children with viral infection aspirin - Reye syndrome
34
What is Buerger disease
necrotizing vasculitis involving digits
35
how does Buerger disease clinically present
- ulceration, gangrene, autoamputation of fingers and toes | - Raynaud phenomenon is often present
36
Buerger disease is associated with what habit?
heavy smoking
37
what is treatment for Buerger disease
smoking cessation
38
What are the small-vessel vasculitis
1. Wegener Granulomatosis 2. Microscopic Polyangiitis 3. Churg-Strauss Syndrome 4. Henoch-Schonlein Purpura
39
What is Wegener Granulomatosis
necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
40
what is the classical presentation of Wegener Granulomatosis
middle-aged male - sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, hematuria due to rapidly progressive glomerulonephritis
41
what lab values go with Wegener Granulomatosis
c-ANCA levels
42
what does biopsy reveal of Wegener Granulomatosis
large necrotizing granulomas with adjacent necrotizing vasculitis
43
what is the treatment for Wegener Granulomatosis
Cyclophosphamide and steroids | -relapses are common
44
what is microscopic polyangiits
necrotizing vasculitis involving multiple organs, especially lungs and kindey
45
clinical presentation of Microscopic polyangiitis
similar to Wegener granulomatosis, | but nasopharyngeal involvement and granulomas are absent
46
what are lab values for microscopic polyangiitis
p-ANCA
47
treatment for microscopic polyangiitis
corticosteroids and cyclophosphamide | relapses are common
48
What is Churg-Strauss syndrome
Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart
49
what is often in people who have Churg-Strauss syndrome
-Asthma and peripheral eosinophilia
50
what are lab values for Churg-Strauss syndrome
p-ANCA
51
What is Henoch-Schonlein Purpura
vasculitis due to IgA immune complex deposition
52
Henoch-Schonlein Purpura is a vasculitis most common found in who
children
53
how does Henoch-Schonlein Purpura clinically present
palapble purpura on buttocks and legs, GI pain and bleeding and hematura ( IgA nephropathy)
54
Henoch-Schonlein Purpura is usually occurs after what infection
upper respiratory tract infection | - because IgA production increased
55
treatment for Henoch-Schonlein Purpura
steroids if severe | - self-limited but may recur