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Cardio Exam 2 > Lec 29 Pathology of Vasculitis > Flashcards

Lec 29 Pathology of Vasculitis Flashcards

1
Q

What is vasculitis?

A

inflammation of blood vessel wall at least some point during course of disease

inflamed vessels –> vascular damage –> end organ damage

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2
Q

What are potential complications of vasculitis?

A

thickening, scarring, narrowing, weakening, thrombosis of affected vessel, aneurysm formation or rupture

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3
Q

How does vasculitis present?

A

constitutional symptoms [fever/myalgias/malaise] PLUS organ specific symptoms

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4
Q

What are 2 major categories of vasculitis by cause?

A

infectious = direct invasion and proliferation in vessel wall by pathogen

non-infectious = not known to be caused by direct vessel wall invasion by pathogen

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5
Q

What are some examples of infectious causes of vasculitis?

A

fungal
bacterial
rickettsial = rocky mountain spotted fever

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6
Q

What usually causes non-infectious causes of vasculitis?

A

most associated wtih immunological mech

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7
Q

What is treatment for infectious vs noninfectious cause of vasculitis?

A

non-infectious –> give immunosuppressive therapy b/c likely immune mediated

infectious = give antibiotic

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8
Q

What is large vessel vasculitis? 2 types?

A

vasculitis that affects aorta and major branches and analogous veins

  • takayasu arteritis
  • giant cell arteritis
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9
Q

What are the 7 categories of vasculitis?

A
  1. large vessel vasculitis
  2. medium vessel vasculitis
  3. small vessel vasculitis
  4. variable vessel vasculitis
  5. single organ vasculitis
  6. vasculitis associated with systemic disease
  7. vasculitis associated with probably etiology
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10
Q

What is medium vessel vasculitis? 2 types?

A

vasculitis that affects main visceral arteries, veins, and initial branches

  • polyarteritis nodosa
  • kawasaki disease
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11
Q

What is small vessel vasculitis?

A

vasculitis that affects intraparenchymal arteries, arterioles, capillaries, venules, veins

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12
Q

What are the 3 immune mech for non-infectious vasculitis?

A
  • immune complex deposition
  • anti-endothelial cell antibodies
  • anti-neutrophil cytoplasmic antibody [ANCA]
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13
Q

What is mech of SLE causing vasculitis?

A

DNA-anti DNA complexes deposit in vessel walls

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14
Q

What is mech of polyarteritis nodosa [PAN] causing vasculitis?

A

in 30% of people have HBsAg-HBsAb complexes deposit

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15
Q

What are some drugs that cause immune complex deposition vasculitis?

A

streptokinase [directly]

penicillin [bound to proteins]

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16
Q

What is anti-neutrophil cytoplasm antibody [ANCA]?

A

ANCA is a circulating antibody that reacts with cytoplasmic antigens in neutrophils and endothelial cells

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17
Q

How are different types of ANCA classified?

A

by intracellular distribution –> either cytoplasmic [c-ANCA] or perinuclear [p-ANCA]

by target antigen –>

  • MPO-ANCA is p-ANCA
  • PR3-ANCA is c-ANCA
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18
Q

What is MPO? Where does MPO ANCA localize?

A

MPO = myeloperoxidase = a lysosomal granlue normally involved in generating oxygen free radicals

localizes to perinculear area = p-ANCA

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19
Q

What is PR3? Where does PR3 ANCA localize?

A

PR3 = proteinase 3 = a neutrophil azurophilic granule constituent

localizes to cytoplasm = c-ANCA

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20
Q

What is clinical use of measuring ANCA level?

A

good for diagnosis or management –> titers reflex disease activity so can monitor for recurrence

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21
Q

What is the major distinction between takayasu arteritis and giant cell arteritis?

A

takayasu = < 50 years

giant cell = > 50 years

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22
Q

How do you distinguish TAK and GCA histopathologically?

A

you can’t

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23
Q

Who usually gets temporal giant cell arteritis?

A

elderly > 50 yo female

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24
Q

What are signs of giant cell arteritis?

A
  • may lead to irreversible blindness due to opthalmic artery occlusion
  • most commonly affects branches of carotid artery
  • high ESR
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25
Q

What are histo features of takayasu/giant cell arteritis

A

granulomatous thickening
- epithelioid macrophages

  • giant cells = fusion of epithelioid macrophages
  • collar of mononuclear cells [lymphocytes]
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26
Q

What artery should you biopsy to diagnose giant cell arteritis? What does negative biopsy tell you?

A

temporal artery biopsy

negative biopsy does not exclude a diagnosis b/c giant cell arteritis is extremely segmental

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27
Q

Who gets takayasu arteritis?

A

asian females < 40 yo

28
Q

What are symptoms of takayasu arteritis?

A

“pulseless disease” = weak upper extremity pulses

fever, nigh sweats, arthritis, myalgias, skin nodules, ocular disturbances

latter sequelae of dialtion/narrowing/occlusion of aorta or branches

29
Q

What are symptoms of giant cell arteritis?

A

have unilateral headache [from temporal artery], jaw claudication

30
Q

What arteries commonly affected by takayasu arteritis?

A

aortic arch and proximal great vessels; commonly left subclavian

31
Q

How do you diagnose takaysu arteritis?

A
  • age of onset < 40 yo
  • signs of limb ischemia
  • decreased pulse in one or both brachial arteries
  • difference in systolic BP between arms
  • bruit in affected subclavian arteries
32
Q

What treatment for giant cell arteritis?

A

corticosteroids with clinical suspicion even if not confirmed by biopsy

33
Q

What treatment of takayasu arteritis?

A

corticosteroids in early stage of inflammation

vascular surgery in later stage for vascular dilation/narrowing etc

34
Q

How does onset of MVV differ from that of LVV?

A

MVV is more acute and necrotizing

inflammatory aneurysms and stenoses are common

LVV = more gradual

35
Q

Who gets polyarteritis nodosa?

step1

A

young adults

30% of pt are hep B positive

36
Q

What are symptoms of polyarteritis nodosa?

step1

A

fever, weight loss, malaise, headache

varied symptoms; depend on which vessels involved

GI: ab main, melena
renal: HTN, renal damage
neuro dysfunction, cutaneous eruptions

37
Q

What is polyarteritis nodosa?

A

necrotizing vasculitis

most frequently involves renal arteries –> arterial HTN and ischemic nephropathy

38
Q

What vessels typically involved in polyarteritis nodosa?

step1

A

most frequently involves renal arteries –> ischemic nephropathy

may involve other organs but usually spares pulmonary circulation

39
Q

Can you get glomerulonephritis from polyarteritis nodosa?

A

no! it does not involve glomerular capillaries

40
Q

What patho features of polyarteritis nodosa?

A
  • immune complex mediated
  • 30% have chronic hep B –> immune complex deposition of HBsAg-HBsAb
  • microaneurysms and spasm on arteriogram
41
Q

What do you use to treat polyarteritis nodosa?

step1

A

corticosteroids

cyclophosphamide

42
Q

What biopsy findings in polyarteritis nodosa?

A
  • transmural inflammation of arterial wall with fibrinoid necrosis [bright pink]
  • sparing of portions of vessel; see all stages of activity in same vessel at same time = ongoing and recurrent insults
  • eventually get chronic inflammation and scarring
43
Q

Who gets kawasaki disease?

step1

A

asian children < 4yo

infants and young children

44
Q

What symptoms of kawasaki disease?

step1

A

acute fever, usually self-limited

+ mucocutaneous lymph node syndrome:
enlarged cervical lymph nodes, conjunctival injection, strawberry tongue, cracked lips, hand-foot erythema, desquamating rash

45
Q

What disease is kawasaki associated with?

step1

A

mucocutaneous lymph node syndrome

46
Q

What vessels primarily affected in kawasaki?

step1

A

coronary arteries –> coronary artery aneurysm, thrombosis –> MI, rupture

47
Q

How do you treat kawasaki?

step1

A

IV immunoglobulin and aspirin

48
Q

What is pathogenesis of kawasaki?

step1

A

due to autoantibodies to endothelial and smooth muscle cells

triggered by infection [usually viral] in genetically susceptible person

49
Q

What is buerger disease?

A

segmental thrombosing vasculitis

in heavy smoker, male, < 40 yo

have intermittent claudication leading to gangrene, auto-amputation of digits, superficial nodular phlebitis

may have raynaud phenomenon

treat with smoking cessation

50
Q

What are the 3 types of ANCA-associated vasculitis?

A

microscopic polyangitis = small vessel vasculitis

granulomatosis with polyangitis = small vessel vasculitis with granulomas

eosinophilic granulomatosis with polyangitis = small vessel vasculitis with granulomas and eosinophiles

51
Q

What is triad of signs that characterizes granulomatosis with polyangitis [wegener’s]?

step1

A
  • necrotizing granulomas in lung and upper airway
  • focal necrotizing vasculitis most prominent in resp organs
  • necrotizing glomerulonephritis = renal disease
52
Q

What is mech of granulomatosis with polyangitis?

step1

A

possible T cell mediated hypersensitivity

PR3-ANCA/c-ANCA

53
Q

What are symptoms of granulomatosis with polyangitis [wegeners]?

step1

A

upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis

lower respiratory tract: hemoptysis, cough, dyspnea

renal: hematuria, red cell casts

54
Q

What is outcome of granulomatosis with polyangitis [wegeners]? treat?

step1

A

die w/in 1 yr if untreated

treat: cyclophsophamide, corticosteroids

55
Q

What is churg strauss syndrome?

step1

A

eosinophilic granulomatosis with polyangitis

  • necrotizing vasculitis
  • granulomas with eosinophils
  • peripheral eosinophilia, asthma, allergic rhinitis
  • assocaited wtih MPO-ANCA/p-ANCA
56
Q

eosinophilic granulomatosis with polyangitis [EGPA] is associated wtih what type of ANCA?

step1

A

MPO-ANCA/p-ANCA

57
Q

granulomatosis with polyangitis [GPA] is associated with what type of ANCA?

step1

A

PR3-ANCA/c-ANCA

58
Q

What symptoms of churg-strauss syndrome?

step1

A

asthma, sinusitis, palpable purpura, peripheral neuropathy [wrist/foot drop]

can also involve heart, GI, kidneys [pauci-immune glomerulonephritis]

high IgE

59
Q

Name the following

  • Arteritis with Fever and mucocutaneous lymph node syndrome
  • Usually
A

kawasaki disease = medium vessel vasculiits

60
Q

Name the following:
- Necrotizing inflammation
Typically involving renal arteries but not glomerular capillaries, hence no glomerulonephritis
- Spares pulmonary vessels
- No ANCA
- 30% associated with chronic HBV infection

A

polyarteritis nodosa

medium vessel vasculitis

61
Q

Name the following:

  • Granulomatous inflammation; frequently involves the temporal artery, used for biopsy diagnosis
  • Usually occurs in patients older than age 50
  • Associated with polymyalgia rheumatica = prox muscle aches and weakness
A

giant cell arteritis

62
Q

Name the following:

  • Granulomatous inflammation, but diagnosis is clinical and radiologic
  • Usually occurring in female patients younger than age 50
  • “Pulseless disease” = b/c of initial manifestion
A

takayasu arteritis

63
Q

Microscopic polyangitis symptoms?

step1

A

necrotizing vasculitis of lungs, kidneys, skin

pauci-immune glomerulonephritis and palpable purpura

similar to granulomatosis with polyangitis but without nasopharyngeal involvemtn

64
Q

What type of ANCA associated wtih microscopic polyangitis?

step1

A

MPO-ANCA/p-ANCA

65
Q

What is treatment fo microscopic polyangitis?

step1

A

cyclophsophamide and corticosteroids

66
Q

What is henoch-schonlein purpura?

A

most common childhood systemic vasculitis

often follows URI

vasculitis seoncary to IgA complex deposition; associated wtih IgA nephropathy

67
Q

What is triad of symptoms henoch-schonlein purpura?

A

skin = palpaple purpura on buttocks/legs

ahtralgias

GI: ab pain, melena, multiple lesions of same age

Cardio Exam 2 (19 decks)

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