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CPR II Exam #1 > Blood Vessels, Part 2 > Flashcards

Blood Vessels, Part 2 Flashcards

1
Q

What does the clinical presentation of vasculitis depend on?

What type of vessel is most commonly affcted?

A

The vascular bed involved (CNS, heart, small bowel, etc.)

Arterioles, capillaries, venules.

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2
Q

Non-infectious vs. infectious vasculitis

A

Non-infectious: immune-mediated (immunosuppressive tx).

Infectious: direct invasion by pathogens.

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3
Q

How can infections indirectly induce non-infectious vasculitis?

A

By generating immune complexes or triggering cross-reactive immune response.

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4
Q

Immune complex vasculitis is a result of:

What diseases/states is it seen in? (3)

A

Deposition of Ag-Ab complexes in vascular walls.

SLE
Drug hypersensitivity (PCN, streptokinase)
Secondary exposure to infectious agent.

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5
Q

30% of Polyarteritis nodosa is associated w/:

A

HBsAg and anti-HBsAg

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6
Q

What are antineutrophil cytoplasmic Abs (ANCA)?

A

A group of Abs that react w/ cytoplasmic enzymes in neutrophil granules, monocytes and endothelial cells.

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7
Q

What are 2 major examples of ANCAs? Their associations?

A

Anti-proteinase-3 (PR3-ANCA) associated w/ polyangiitis

Anti-myeloperoxidase (MPO-ANCA) induced by RX propylthiouracil.

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8
Q

What do ANCA activate?

A

Neutrophils which release ROS

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9
Q

Why are ANCA considered “pauci-immune”?

A

Because the ANCA Ab directed against cellular constituents and do NOT form circulating immune complexes.

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10
Q

Giant cell arteritis and aortitis is most common vasculitis among which patients?

What is the presentation?

A

Older patients.

Constitutional complaints along w/ facial pain and headache.

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11
Q

What is Segmental Transmural necrotizing inflammation?

A

WBC inflammation/necrosis w/ a predilection for branch points. It spans the entire arterial wall.

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12
Q

What is the course of PAN?

A

Course is remitting and episodic.

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13
Q

What ANCA is microscopic polyangiitis associated with?

A

MPO-ANCA

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14
Q

What else is Microscopic polyangiitis known as? (2)

A

Leukocyte vasculitis or Hypersensitivity vasculitis

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15
Q

How does myocardial vessel vasospasm occur?

What is the outcome? (2)

A

Excessive vasoconstriction of myocardial as. or arterioles (cardiac Raynaud) and may cause ischemia or infarct. Usually occurs as a result of high levels of vasoactive mediators (epi, pheochromocytoma, cocaine).

Sudden cardiac death or Takotsubo cardiomyopathy (broken heart sybdrome assoc. w/ emotional distress).

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16
Q

What causes varicose veins?

A

Abn. dilation of veins w/ valvular incompetence, secondary to sustained intraluminal pressure.

Rarely embolizes.

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17
Q

What causes esophageal varices?

3 classes:

A

Portal HTN (often cirrhosis) opens a shunt to direct blood to veins at gastroesophageal junction.

Esophageal varices, hemorrhoids, caput medusae.

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18
Q

Thrombophlebitis is:

Includes which vessels?

A

Venous thrombosis and inflammation.

Almost always in deep vs. of leg.

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19
Q

What can cause Migratory thrombophlebitis (4)

A

Cancer pts. from a paraneoplastic syndrome.
Mucin-producing adenocarcinomas.
Spontaneous thromboses that form and resolve, etc.
Adenocarcinomas of lung, ovary, pancreas.

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20
Q

SVC syndrome cause:

Clinical picture:

A

Neoplasms compress or invade SVC.

Complex, but marked dilation of vs. in head, neck and arms w/ cyanosis.
Pulm. vessels can be compressed and lead to respiratory distress.

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21
Q

What 2 main cancers can lead to IVC syndrome?

Clinical picture?

A

Hepatocellular carcinoma and renal cell carcinoma tend to grow in the IVC and can lead all the way to the RA.

LE edema, venous distension in lower abdomen, massive proteinuria due to renal v. involvement*.

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22
Q

Most common agent in lymphangitis?

A

Grp A beta-hemolytic strep

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23
Q

Primary lymphedema cause:

Secondary or obstructive lymphedema cause:

A

Isolated congenital defect (Milroy DZ).

Blockage of normal lymph flow.

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24
Q

What is peau d’ orange?

A

Used to describe the skin overlying breast cancer where lymph is clogged with tumor cells.

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25
Q

Markers used to DX neoplasia of the venous system?

A

Endothelial cell-specific markers like CD31 or vWF.

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26
Q

Nevus flammeus means:

A

Birthmark

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27
Q

Cavernous hemangioma

A

Irregular dilated vascular channels make a lesion w/ indistint border.
More likely to involve deep tissue/bleed.

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28
Q

Pyogenic granuloma

A

Type of capillary hemangioma (neither pyogenic or hemangioma).
Rapidly growing, often in oral mucosa.
An example is granuloma gravidarum (pregnancy tumor) in gingiva of pregnant women.

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29
Q

Glomus tumor

A

Benign tumors from glomus bodies most often in distal fingers.
SM origin rather than endothelial.
Very painful.

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30
Q

Bacillary angiomatosis is from what bug?

Common in which pts.?

What is a classic appearance?

How can the bacteria be identified? (2)

What is an effective Tx?

A

Bartonella bacilli.

Immunocompromised pts.

Form red papules due to *proliferation of capillaries w/ plump endothelial cells.

PCR or visualized with a *Warthin-Starry stain.

Macrolide abx.

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31
Q

2 intermediate-grade (bordeline) vascular tumors

A

Epithelioid hemangioendothelioma

Kaposi sarcoma

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32
Q

Epithelioid hemangioendothelioma has what classic appearance at the cellular level?

What is the progression of DZ?

A

Neoplastic endothelial cells are plump and cuboidal and vascular channels may be hard to recognize.

Variable clinical behavior, but mets 20-30% of the time.

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33
Q

4 distinct clinical forms of KS

A
  1. AIDS-assoc. (most common AIDS related tumor)
  2. Classic KS: older men from middle east, Mediterranean. Tumors on skin.
  3. Endemic African KS: Pts < 40 yo. Can involve cervical LNs.
  4. Transplant-assoc. KS: assoc. w/ T cell immunosuppression. Can spread to LNs and viscera.
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34
Q

Angiosarcoma is malignant. Which age group is most affected?

What can cause it? (2)

Common sites: (4)

What is the prognosis at 5 yrs.?

A

Older pts. M=F.

Can be induced by radiation exposure. Can arise in setting of lymphedema.

Most common sites include skin, soft tissue, breast and liver.

5 yr. survival around 30%.

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35
Q

What is associated with hepatic angiosarcoma? (4)

A

Arsenic
Pesticides
Thorotrats
Polyvinyl chloride

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36
Q

3 ways to intervene in vascular defects via stenting

A

Balloon angioplasty
Coronary stent
Drug-eluting stents

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37
Q

Vascular replacement includes synthetic or autologous vascular grafts. What is an issue w/ synthetic large bora grafts?

A

They will work for aorta, but won’t work for smaller vessels (i.e. coronary as.) due to thrombosis or intimal hyperplasia at the junction of graft and native vasculature.

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38
Q

What vessels are used for small bore grafts? (2)

What are their patencies at 10 yrs.?

A

Saphenous vs. or left internal mammary as.

Saphenous vs. 50% patency at 10 yrs.
LIM as. 90% patency at 10 yrs.

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39
Q

Giant cell arteritis and aortitis: patient population

A

Older patients

40
Q

Giant cell arteritis and aortitis: presenting symptoms

A

Facial pain

Headache

41
Q

Giant cell arteritis and aortitis: major pathology

A

T-cell mediated (CD4+ > CD8+) inflammation of the temporal aa.

42
Q

Giant cell arteritis and aortitis: histological features (3)

A

Multinucleated Giant cells
Fragmented elastic lamina and intimal thickening
Patchy and focal sites of involvement

43
Q

Giant cell arteritis and aortitis: unique feature

A

Involvement of the ophthalmic a. may cause double vision.

44
Q

Takayasu arteritis: patient population

A

Younger (< 50 y/o)

45
Q

Takayasu arteritis: presenting symptoms

A

Weak pulses of the UE and UE BP.

46
Q

Takayasu arteritis: major pathology

A

Granulomatous vasculitis of medium and larger aa. (pulmonary, coronary, renal aa.).

47
Q

Takayasu arteritis: unique features (2)

A

Ocular disturbances may ensue

“Pulseless disease”

48
Q

PAN: presenting population

A

Young adults

49
Q

PAN: presenting symptoms

A

Accelerating HTN
Abdominal pain and bloody stool
Diffuse myalgias
Peripheral neuritis

50
Q

PAN: major pathology

A

Systemic vasculitis of renal, heart, liver and GI tract vessels. (NO pulm.)

51
Q

PAN: first sign of disease

A

Ulcerations, infarcts, ischemia and hemorrhage.

52
Q

PAN: treatment

A

Immunosuppressive therapy

53
Q

PAN: unique features (2)

A

1/3 of patients have Hep B

Segmental transmural necrotizing inflammation ensues w/ a predilection for branch points

54
Q

Kawasaki disease: patient population

A

Infants and small children (< 4 y/o)

55
Q

Kawasaki disease: presenting symptoms (3)

A

Erythema of conjunctive, oral mucosa, palms and soles
Desquamative rash
Cervical LN enlargement

56
Q

Kawasaki disease: major pathology

A

Coronary a. -> aneurysm -> thrombosis/rupture -> acute MI

57
Q

Kawasaki disease: treatment

A

IVIg and Aspirin to lower risk of MI

58
Q

Microscopic polyangiitis: presenting symptoms

A 
Hemoptysis
Hematuria and proteinuria
Bowel pain/bleeding
Myalgias
Palpable cutaneous purpura
59
Q

Microscopic polyangiitis: major pathology

A

Necrotizing vasculitis involving arterioles, capillaries and venules.

60
Q

Microscopic polyangiitis: most common sites of involvement (2)

A

Renal glomeruli

Lung capillaries

61
Q

Microscopic polyangiitis: unique features (2)

A

Associated w/ MPO-ANCA

Segmental necrotizing inflammation w/ fibrinoid necrosis

62
Q

Churg-Strauss syndrome: presenting symptoms

A 
Asthma
Allergic rhinitis
Hypereosinophilia
Lung infiltrates
Palpable purpura
63
Q

Churg-Strauss syndrome: major pathology

A

Small vessel necrotizing vasculitis w/ inflammation that includes eosinophils and granulomas.

64
Q

Churg-Strauss syndrome: organ systems involved

A

Skin (purpura)
GI tract (GI bleed)
Kidneys (renal DZ)
Heart (cardiomyopathy)

65
Q

Churg-Strauss syndrome: most common COD

A

Heart pathology for approx. 50%

66
Q

PAN: most common COD

A

Renal disease

67
Q

Behcet disease: presenting symptoms

A

Triad: oral ulcers, genital ulcers, uveitis

68
Q

Behcet disease: major pathology

A

Vasculitis of small-medium vessels w/ neutrophilic inflammation

69
Q

Behcet disease: disease mortality is related to…

A

Neurologic involvement or rupture of aneurysms.

70
Q

Behcet disease: treatment (2)

A

Steroids

TNF-antagonists

71
Q

Behcet disease: unique cell marker association

A

HLA-B51

72
Q

Granulomatosis with polyangiitis: patient population

A

M>F with average of 40 y/o

73
Q

Granulomatosis with polyangiitis: presenting symptoms

A

Pneumonitis
Sinusitis
Renal disease
Nasopharyngeal ulceration

74
Q

Granulomatosis with polyangiitis: major pathology

A

Necrotizing vasculitis of URT and LRT.
May include focal necrotizing, often crescentic, glomerulonephritis.

It is a form of T cell mediated hypersensitivity response to innocuous microbes/insults.

75
Q

Granulomatosis with polyangiitis: treatment (3)

A

Steroids
Cyclophosphamide
TNF-antagonists

76
Q

Granulomatosis with polyangiitis: course

A

Chronic relapsing and remitting

77
Q

Granulomatosis with polyangiitis: morphology in URT and LRT

A

URT: geographic patterns of central necrosis.

LRT: cavitory lesions.

78
Q

Granulomatosis with polyangiitis: unique ANCA association

A

PR3-ANCA

79
Q

Thromboangiitis obliterans: patient population

A

Heavy smokers, < 35 y/o, often Israeli, Indian and Japanese descent.

80
Q

Thromboangiitis obliterans: presenting symptoms (3)

A

Cold-induced Raynaud’s
Leg pain at rest
Superficial nodular phlebitis

Chronic extremity ulcerations may lead to frank gangrene

81
Q

Thromboangiitis obliterans: major pathology

A

Segmental, thrombosing, acute and chronic vasculitis of small and medium vessels, primarily the tibial and radial aa.

82
Q

Thromboangiitis obliterans: unique features (2)

A

May produce a thrombus with small “microabscesses”.

Most affected patients have a hypersensitivity to injected tobacco products.

83
Q

What 2 arteries are affected in Thromboangiitis obliterans?

A

Radial a.

Tibial a.

84
Q

What DZ is associated w/ PR3-ANCA?

A

Granulomatosis with polyangiitis

85
Q

Steroids
Cyclophosphamide
TNF-antagonists

Are treatments for?

A

Granulomatosis with polyangiitis

86
Q

Steroids
TNF-antagonists

Are treatments for?

A

Behcet DZ

87
Q

Skin (purpura)
GI tract (GI bleed)
Kidneys (renal DZ)
Heart (cardiomyopathy)

Are involved in which disease?

A

Churg-Strauss

88
Q

Triad: oral ulcers, genital ulcers, uveitis

A

Behcet DZ

89
Q

Which disease is associated w/ MPO-ANCA?

A

Microscopic polyangiitis

90
Q

Which disease involves systemic vasculitis of renal, heart, liver and GI tract vessels?

A

PAN

91
Q

What is a major symptom of IVC syndrome?

A

Massive proteinuria

92
Q

Erythema of conjunctive, oral mucosa, palms and soles
Desquamative rash
Cervical LN enlargement

Are SX of?

A

Kawasaki DZ

93
Q

Which DZs have palpable purpura? (2)

A

Microscopic polyangiitis

Churg-Strauss

94
Q

What are 3 major arteries involved in Takayasu arteritis?

A

Pulm. a.
Coronary a.
Renal a.

95
Q

Renal glomeruli
Lung capillaries

Are involved in which disease?

A

Microscopic polyangiitis

96
Q

Eosinophilia, Rhinitis, Asthma

A

Churg-Strauss

97
Q 
Hemoptysis
Hematuria and proteinuria
Bowel pain/bleeding
Myalgias
Palpable cutaneous purpura
A

Microscopic polyangiitis

CPR II Exam #1 (30 decks)

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