Vascular Pathology 2 - Vasculitis, Vessel Hyperreactivity, Veins and Lymphatics, Vascular Tumors, Flashcards

1
Q

The 2 classifications of vasculidities and the reason distinction is important for tx.

A

Infectious (pathogenic) and noninfectious (immunologic injury).
Noninfectious can be treated with immunosuppressive therapy.

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2
Q

What is immune complex vasculitis?

A

Deposition of antigen-antibody complexes in vascular walls&raquo_space; inciting inflammatory reaction

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3
Q

What cases can immune complex vasculitis be seen in?

A

Systemic immunologic Diseases - SLE
Drug hypersensitivity
Secondary exposure of infectious agent

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4
Q

What are antineutrophil cytoplasmic antibodies?

A

ANCA (antibodies) reactant with cytoplasmic granules found in neuts, monocytes, and endothelial cells.

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5
Q

What was formerly called c-ANCA?

What was formerly called p-ANCA?

A

Anti-proteinase3 (Wegners Granulomatosis)

Anti-myeloperoxidase (Microscopic Polyangiitis)

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6
Q

What do ANCAs do?

A

Activate neuts, which release ROS.

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7
Q

Name the (2) large vessel vasculitides - both Granulomatous Disease

A

Giant Cell Arteritis

Takayasu Arteritis

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8
Q

Name the medium vessel vascilitides (3).

A

1) Immune complex mediated - PAN
2) Anti-endothelial cell bodies - Kawasaki disease.
3) Bechet Disease (small and medium vessels)

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9
Q

Name the small vessel vasculitides (4)

A

1) Microscopic polyangiitis
2) Churg-Strauss Syndrome
3) Thromboangiitis Obliterans (small and medium vessels)
4) Bechet Disease (small and medium vessels)

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10
Q

What am I?
An elderly female presents with HA ad unilateral temporal pain with firm nodules on palpation. She may complain of blindness. You see giant cell granuloma. Joints may hurt, but not caused by arthritis.

A

Giant cell arteritis

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11
Q

What is the most common vasculitis among older patients?

A

Giant cell arteritis

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12
Q

What large vessel arteritis is associated with fragmented elastic lamina and medial/intimal thickening of the temporal arteries?

A

Giant cell arteritis

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13
Q

What tx does giant cell arteritis respond well to?

A

Steroids

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14
Q

What large vessel arteritis is associated with fragmented elastic lamina and medial/intimal thickening of the aortic arch (esp. carotid and subclavian)?

A

Takayasu arteritis

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15
Q

What age group does Takayasu Arteritis affect?

A

People less than 50yo.

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16
Q

What arteritis may present with weak pulse and low BP in upper extremities?

A

Takayasu arteritis

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17
Q

What is the “pulseless disease”?

What is the main artery involved, what other 3 may also be involveD?

A

Main - Aortic arch

Other - Pulmonary, coronary, renal arteries

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18
Q

What arteritis am I?

1/3 of patients with this have chronic hep B, usually young adult.

A

PAN

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19
Q

What vessels are affected in PAN, what vessels are not affected?

A

Involved: Renal vessels > heart > liver > GI

Not involved: Pulmonary vessels

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20
Q

Tx for PAN

A

immunosuppressive therapy

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21
Q

What am I?

A 3yo with an MI, rash, and cervical LN involvement. What else is this called?

A

Kawasaki Disease

“Mucocutanous lymph node syndrome”

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22
Q

What artery does Kawasaki often involve?

A

Coronary arteries

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23
Q

What 2 things are given to children with Kawasaki to lower risk of coronary event?

A

IV-Ig and Aspirin

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24
Q

What symptoms (other than coronary involvement) are associated with Kawasaki

A

Skin.
Erythema of conjuctiva, oral mucosa, palms, and soles
Rash
Cervical LN enlargement

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25
Q

What vessels does microscopic polyangiitis most often affect?

A

Renal glom and lung capillaries

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26
Q

What ANCA is microscopic polyangiitis associated with?

A

MPO-ANCA

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27
Q

What is another name for microscopic polyangiitis?

A

leukocytoclastic vasculitis

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28
Q

Describe the necrosis in microscopic polyangiitis.

A

Segmental necrotizing inflammation with fibrinoid necrosis and many apoptotic neuts.

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29
Q

What 3 main things is Churg-Strauss Syndrome associated with?

A

Allergic rhinitis, asthma, and hypereosinophila

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30
Q

Associate eosinophilia and granulomas with what?

Without these two associations, what does this disease look like?

A

Churg-Strauss (with eosino and granulomas)

PAN (without eosino and granulomas)

31
Q

What vasculitis do you associate with HLA-B51?

A

Bechet Disease

32
Q

What three PE findings are associated with Bechet Disease?

A

Aphthous ulcers of oral cavity
Genital Ulcers
Uveitis

33
Q

What am I?

Necrotizing granulomas predominantly in upper and/or lower Respiratory Tracts.

A

Granulomatosis with polyangiitis (Wegner)

34
Q

What is associated with PR3-ANCA?

A

Granulomatosis with polyangiitis

35
Q

Granulomatosis with polyangiitis is treated how?

A

immunosuppressive therapy

36
Q

What sex and age group most often affected in Granulomatosis with polyangiitis?

A

Males > Females

Avg age of 40yo

37
Q

What other clinical features are associated with granulomatosis polyangiitis?

A

Other: persistent pneumonitis, sinusitis, arenla disease, nasopharyngeal ulceration

38
Q

Tibial and radial arteries are associated with what vasculitis?

A

Thromboangiitis obliterans

39
Q

Smoker, YA = what vasculitis?

A

Thromboangiitis obliterans

40
Q

What clinical features can result from thromboangiitis obliterans (3)?

A

1) Gangrene due to chronic ulcerations.
2) Pain due to inflammations extending to adjacent veins and nerves.
3) Vascular insufficiency in extremities

41
Q

What are the two types of Raynaud’s? How are they differentiated?

A
Primary = cold induced vasospasm = white and symmetric
Secondary = due to arterial disease  = cyanosis = blue and asymmetric
42
Q

What arterial diseases can cause secondary Raynaud’s?

A

SLE
SCLERODERMA
Thromboangiitis obliterans

43
Q

Demographic that would get primary Raynauds?

A

Young women exposed to cold temps

44
Q

Which Raynaud’s is benign, which is progressive?

A

Primary is benign.

Secondary is progressive.

45
Q

What can cause myocardial vessel vasospasm?

A

Endogenous - epinephrine

Exogenous - cocaine

46
Q

What happens in myocardial vessel vasospasm?

A

Artery/arteriole vasoconstriction that can result in ischemia or infarct.

47
Q

What four locations does dilation of veins most often occur?

Which rupture is most fatal?

A

Superficial leg veins - varicose veins
Esophagus - esophageal varices Most fatal
Anus - hemorrhoids
Periumbilicus - Caput Medusa

48
Q

Portal HTN (often due to cirrhosis) is associated with formation of what?

A

Esophageal varices

49
Q

What two things increase risk of DVT (thrombophlebitis)?

A
  1. Prolonged inactivity and immobilization

2. Systemic hypercoagulability - i.e. Factor 5 Leiden deficiency

50
Q

What is the most serious potential consequence of DVTs?

A

Pulmonary embolism

51
Q

What are mucin-producing adenocarcinomas associated with?

A

Migratory thrombophlebitis (trosseau sign).

52
Q

What are the three most commonly associated locations for the migratory-thrombophlebitis-causing-adenocarcinoma?

A

Lung, ovary, pancreas

53
Q

What are the three types of hemangiomas?

A

(1) Capillary Hemanigioma –> (2) Pyogenic Hemangioma (Lobular Capillary Hemangioma)
(3) Cavernous Hemangioma

54
Q

Which hemangioma is tightly packed and well circumscribed? What type of vessel?

A

Capillary Hemangioma in thin walled capillaries

55
Q

Which has irregular vascular channels with indistinct borders? Where is it located?

A

Cavernous Hemangioma in deep tissues.

56
Q

What hemangioma is associated with oral mucosa?

A

Pyogenic Granuloma (lobular capillary heamngioma)

57
Q

Why is pyogenic granuloma misnamed?

A

It’s not pyogenic and not a granuloma - it’s a type of capillary hemangioma

58
Q

What are the two types of lymphangiomas?

A

1) Simple

2) Cavernous

59
Q

How do you differentate a capillary hemangioma from a simple lymphangioma?

A

Lack of RBCs in lymphangioma

60
Q

You see a Female child with large, lymphatic nodules in the neck or axilla area. What lymphangioma is this and what should you also look for?

A
Cavernous lymphangioma (cystic hygroma)
Turner Syndrome
61
Q

Person presents with a PAINFUL tumor in the distal fingers - what type of benign vascular tumor is this?

A

Glomus tumor

62
Q

Where do glomus tumors originate?

A

Smooth muscle origin (not endothelial).

63
Q

What vascular tumor is associated with (gram-negative) Bartonella bacilli? What is used to visualize this bacteria?

A

Bacillary Angiomatosis visualized with Warthin-Starry stain.

64
Q

Tx for Bacillary Angiomatosis?

A

Macrolide antibiotics are effective.

65
Q

What is this vascular tumor?
An immunocompromised person presents with lesions that are localized, red papules. Microscopically, there is a proliferation of capillaries with plump endothelial cells.

A

Bacillary angiomatosis

66
Q

What intermediate vascular tumor has vascular channels that are difficult to see?

A

Epithelioid hemangioendothelioma

67
Q

What vascular tumor is associated with HHV-8?

A

KS

68
Q

What KS is localized to the skin? What demographics is this type most common in?

A

Classic KS - Older men of Middle Eastern, Mediterranean, or Eastern European descent.

69
Q

What KS is associated with people less than 40yo and children who don’t wear shoes?

A

Endemic African KS

70
Q

What KS is associated with T cell immunosuppression? How do you treat this type of KS?

A

Transplant-associated KS

Tx: reduce immunosupression

71
Q

What KS’s are in both the LNs and viscera?

A

AIDS-associated KS

Transplant-associated KS

72
Q

What are the most common sites of angiosarcoma?

Most common demographic?

A

skin, soft tissue, breast, liver

M=F, older

73
Q

What vascular tumor is associated with endothelial marker CD31?

A

Angiosarcoma

74
Q

What vascular tumor is associated with “glistening tissue”?

A

Angiosarcoma