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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
What are histo features of takayasu/giant cell arteritis
granulomatous thickening - epithelioid macrophages - giant cells = fusion of epithelioid macrophages - collar of mononuclear cells [lymphocytes]
26
What artery should you biopsy to diagnose giant cell arteritis? What does negative biopsy tell you?
temporal artery biopsy negative biopsy does not exclude a diagnosis b/c giant cell arteritis is extremely segmental
27
Who gets takayasu arteritis?
asian females < 40 yo
28
What are symptoms of takayasu arteritis?
"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
What are symptoms of giant cell arteritis?
have unilateral headache [from temporal artery], jaw claudication
30
What arteries commonly affected by takayasu arteritis?
aortic arch and proximal great vessels; commonly left subclavian
31
How do you diagnose takaysu arteritis?
- 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
What treatment for giant cell arteritis?
corticosteroids with clinical suspicion even if not confirmed by biopsy
33
What treatment of takayasu arteritis?
corticosteroids in early stage of inflammation vascular surgery in later stage for vascular dilation/narrowing etc
34
How does onset of MVV differ from that of LVV?
MVV is more acute and necrotizing inflammatory aneurysms and stenoses are common LVV = more gradual
35
Who gets polyarteritis nodosa? step1
young adults | 30% of pt are hep B positive
36
What are symptoms of polyarteritis nodosa? step1
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
What is polyarteritis nodosa?
necrotizing vasculitis | most frequently involves renal arteries --> arterial HTN and ischemic nephropathy
38
What vessels typically involved in polyarteritis nodosa? step1
most frequently involves renal arteries --> ischemic nephropathy may involve other organs but usually spares pulmonary circulation
39
Can you get glomerulonephritis from polyarteritis nodosa?
no! it does not involve glomerular capillaries
40
What patho features of polyarteritis nodosa?
- immune complex mediated - 30% have chronic hep B --> immune complex deposition of HBsAg-HBsAb - microaneurysms and spasm on arteriogram
41
What do you use to treat polyarteritis nodosa? step1
corticosteroids | cyclophosphamide
42
What biopsy findings in polyarteritis nodosa?
- 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
Who gets kawasaki disease? step1
asian children < 4yo | infants and young children
44
What symptoms of kawasaki disease? step1
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
What disease is kawasaki associated with? step1
mucocutaneous lymph node syndrome
46
What vessels primarily affected in kawasaki? step1
coronary arteries --> coronary artery aneurysm, thrombosis --> MI, rupture
47
How do you treat kawasaki? step1
IV immunoglobulin and aspirin
48
What is pathogenesis of kawasaki? step1
due to autoantibodies to endothelial and smooth muscle cells triggered by infection [usually viral] in genetically susceptible person
49
What is buerger disease?
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
What are the 3 types of ANCA-associated vasculitis?
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
What is triad of signs that characterizes granulomatosis with polyangitis [wegener's]? step1
- necrotizing granulomas in lung and upper airway - focal necrotizing vasculitis most prominent in resp organs - necrotizing glomerulonephritis = renal disease
52
What is mech of granulomatosis with polyangitis? step1
possible T cell mediated hypersensitivity PR3-ANCA/c-ANCA
53
What are symptoms of granulomatosis with polyangitis [wegeners]? step1
upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis lower respiratory tract: hemoptysis, cough, dyspnea renal: hematuria, red cell casts
54
What is outcome of granulomatosis with polyangitis [wegeners]? treat? step1
die w/in 1 yr if untreated treat: cyclophsophamide, corticosteroids
55
What is churg strauss syndrome? step1
eosinophilic granulomatosis with polyangitis - necrotizing vasculitis - granulomas with eosinophils - peripheral eosinophilia, asthma, allergic rhinitis - assocaited wtih MPO-ANCA/p-ANCA
56
eosinophilic granulomatosis with polyangitis [EGPA] is associated wtih what type of ANCA? step1
MPO-ANCA/p-ANCA
57
granulomatosis with polyangitis [GPA] is associated with what type of ANCA? step1
PR3-ANCA/c-ANCA
58
What symptoms of churg-strauss syndrome? step1
asthma, sinusitis, palpable purpura, peripheral neuropathy [wrist/foot drop] can also involve heart, GI, kidneys [pauci-immune glomerulonephritis] high IgE
59
Name the following - Arteritis with Fever and mucocutaneous lymph node syndrome - Usually
kawasaki disease = medium vessel vasculiits
60
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
polyarteritis nodosa medium vessel vasculitis
61
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
giant cell arteritis
62
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
takayasu arteritis
63
Microscopic polyangitis symptoms? step1
necrotizing vasculitis of lungs, kidneys, skin pauci-immune glomerulonephritis and palpable purpura similar to granulomatosis with polyangitis but without nasopharyngeal involvemtn
64
What type of ANCA associated wtih microscopic polyangitis? step1
MPO-ANCA/p-ANCA
65
What is treatment fo microscopic polyangitis? step1
cyclophsophamide and corticosteroids
66
What is henoch-schonlein purpura?
most common childhood systemic vasculitis often follows URI vasculitis seoncary to IgA complex deposition; associated wtih IgA nephropathy
67
What is triad of symptoms henoch-schonlein purpura?
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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