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
Q

melena

A

dark sticky feces containing partly digested blood

26
Q

what are abnormal values associated with polyarteritis nodosa

A

associated with serum HBsAg

hepatitis B surface antigen

27
Q

How do lesions present in polyarteritis nodosa

A
  • varying stages present at once
  • Early: transmural inflammation with fibrinoid necrosis
  • early then heals making ‘string-of-pearls’ appearance
28
Q

treatment for polyarteritis nodosa

A

corticosteroids and cyclophosphamide

- fatal if not treated

29
Q

Kawasaki disease classically affects what group of people

A

asian children less than 4 yrs old

30
Q

how does Kawasaki present

A

non-specific signs: fever, conjunctivitis, erythematous rash of palms and soles, enlarged cervical lymph node

31
Q

what part of the body is commonly involved with Kawasaki disease? what does this lead to

A

coronary artery

  1. thrombosis with myocardial infarction
  2. aneurysm with rupture
32
Q

what is the treatment for Kawasaki

A

aspirin and IVIG

disease is self-limited

33
Q

what is the caution with giving aspirin with an asian child presenting with Kawasaki

A

clinical features of Kawasaki similar to viral disease

  • not suppose to give children with viral infection aspirin
  • Reye syndrome
34
Q

What is Buerger disease

A

necrotizing vasculitis involving digits

35
Q

how does Buerger disease clinically present

A
  • ulceration, gangrene, autoamputation of fingers and toes

- Raynaud phenomenon is often present

36
Q

Buerger disease is associated with what habit?

A

heavy smoking

37
Q

what is treatment for Buerger disease

A

smoking cessation

38
Q

What are the small-vessel vasculitis

A
  1. Wegener Granulomatosis
  2. Microscopic Polyangiitis
  3. Churg-Strauss Syndrome
  4. Henoch-Schonlein Purpura
39
Q

What is Wegener Granulomatosis

A

necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys

40
Q

what is the classical presentation of Wegener Granulomatosis

A

middle-aged male
- sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, hematuria due to rapidly progressive glomerulonephritis

41
Q

what lab values go with Wegener Granulomatosis

A

c-ANCA levels

42
Q

what does biopsy reveal of Wegener Granulomatosis

A

large necrotizing granulomas with adjacent necrotizing vasculitis

43
Q

what is the treatment for Wegener Granulomatosis

A

Cyclophosphamide and steroids

-relapses are common

44
Q

what is microscopic polyangiits

A

necrotizing vasculitis involving multiple organs, especially lungs and kindey

45
Q

clinical presentation of Microscopic polyangiitis

A

similar to Wegener granulomatosis,

but nasopharyngeal involvement and granulomas are absent

46
Q

what are lab values for microscopic polyangiitis

A

p-ANCA

47
Q

treatment for microscopic polyangiitis

A

corticosteroids and cyclophosphamide

relapses are common

48
Q

What is Churg-Strauss syndrome

A

Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart

49
Q

what is often in people who have Churg-Strauss syndrome

A

-Asthma and peripheral eosinophilia

50
Q

what are lab values for Churg-Strauss syndrome

A

p-ANCA

51
Q

What is Henoch-Schonlein Purpura

A

vasculitis due to IgA immune complex deposition

52
Q

Henoch-Schonlein Purpura is a vasculitis most common found in who

A

children

53
Q

how does Henoch-Schonlein Purpura clinically present

A

palapble purpura on buttocks and legs, GI pain and bleeding and hematura ( IgA nephropathy)

54
Q

Henoch-Schonlein Purpura is usually occurs after what infection

A

upper respiratory tract infection

- because IgA production increased

55
Q

treatment for Henoch-Schonlein Purpura

A

steroids if severe

- self-limited but may recur