Card - Path (Part 6: Vascular Disorders) Flashcards

Pg. 294-297 in First Aid 2014 Pg. 276-278 in First Aid 2013 Sections include: -Raynaud's phenomenon -Vasculitis -Vascular tumors

1
Q

What is Raynaud’s phenomenon? What is the mechanism, and to what is it a response?

A

Decreased blood flow to the skin due to arteriolar vasospasm in response to cold temperature or emotional stress

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2
Q

Where is Raynaud’s phenomenon most often found on the body? What type of vessels does it affect?

A

Most often in the fingers and toes; Affects small vessels

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3
Q

What are the 2 different names given to Raynaud’s phenomenon, and in what contexts?

A

(1) Raynaud’s disease: primary (idiopathic) (2) Raynaud’s syndrome: secondary to a disease process (such as mixed connective tissue disease, SLE, or CREST [limited form of systemic sclerosis] syndrome)

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4
Q

What is Raynaud’s syndrome? What are some disease processes with which it may be associated?

A

Raynaud’s phenomenon (decreased blood flow to skin due to arteriolar vasospasm in response to cold temperature of emotional stress) secondary to a disease process (such as mixed connective tissue disease, SLE, or CREST [limited form of systemic sclerosis] syndrome)

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5
Q

Give 9 examples of vascular tumors.

A

(1) Strawberry hemangioma (2) Cherry hemangioma (3) Pyogenic granuloma (4) Cystic hygroma (5) Glomus tumor (6) Bacillary angiomatosis (7) Angiosarcoma (8) Lymphangiosarcoma (9) Kaposi sarcoma

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6
Q

What is a Strawberry hemangioma? When does it appear, and what is its incidence? How does it develop and/or resolve over time?

A

Benign capillary hemangioma of infancy. Appears in first few weeks of life (1/200 births); grows rapidly and regresses spontaneously at 5 to 8 years old

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7
Q

What is a Cherry hemangioma? Does it regress? How does it change with age?

A

Benign capillary hemangioma of the elderly. Does not regress. Frequency increased with age.

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8
Q

What defines a pyogenic granuloma? With what 2 conditions is it associated?

A

Polypoid capillary hemangioma that can ulcerate and bleed. Associated with trauma and pregnancy.

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9
Q

What is a cystic hygroma? With what condition is it associated?

A

Cavernous lymphangioma of the neck. Associated with Turner syndrome.

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10
Q

What is a glomus tumor? From where does it arise?

A

Benign, painful, red-blue tumor under fingernails. Arises from modified smooth muscle cells of glomus body.

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11
Q

What defines bacillary angiomatosis? What causes this? For what is this frequently mistaken?

A

Benign capillary skin papules found in AIDS patients. Caused by Bartonella henselae infections. Frequently mistaken for Kaposi sarcoma.

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12
Q

What is angiosarcoma? Where does it typically occur, and in what patient population?

A

Rare blood vessel malignancy typically occurring in the head, neck, and breast areas. Usually in elderly, on sun-exposed areas.

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13
Q

With what 2 exposures is angiosarcoma associated?

A

Associated with radiation therapy and arsenic exposure.

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14
Q

Briefly discuss the progression and resection of angiosarcoma.

A

Very aggressive and difficult to resect due to delay in diagnosis.

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15
Q

What is lymphangiosarcoma, and with what is it associated? Give an example of a context in which this may occur.

A

Lymphatic malignancy associated with persistent lymphedema (e.g., post-radical mastectomy)

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16
Q

What is Kaposi sarcoma, and where in the body is it most commonly found? Where else it found?

A

Endothelial malignancy most commonly of the skin, but also mouth, GI tract, and respiratory tract

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17
Q

With what 2 pathogens is Kaposi sarcoma associated?

A

Associated with HHV-8 and HIV

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18
Q

For what is Kaposi sarcoma frequently mistaken?

A

Frequently mistaken for bacillary angiomatosis

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19
Q

What are the 3 major categories of vasculitis?

A

(1) Large-vessel vasculitis (2) Medium-vessel vasculitis (3) Small-vessel vasculitis

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20
Q

What are 2 types of large-vessel vasculitis?

A

(1) Temporal (giant cell) arteritis (2) Takayasu arteritis

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21
Q

What is another name for temporal arteritis? What patient population is generally affected by it?

A

Temporal (giant cell) arteritis; Generally elderly females

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22
Q

What are 3 symptoms/signs in the presentation of Temporal (giant cell) arteritis? Where applicable, give the cause. With what other condition is Temporal (giant cell) arteritis associated?

A

(1) Unilateral headache (temporal artery), (2) Jaw claudication (3) May lead to irreversible blindness due to opthalmic artery occlusion.; Associated with polymyalgia rheumatica

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23
Q

Which vessels are most commonly affected by Temporal (giant cell) arteritis?

A

Most commonly affects branches of carotid artery

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24
Q

What are 2 histological/lab findings that characterize Temporal (giant cell) arteritis?

A

(1) Focal granulomatous inflammation (2) High ESR

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25
Q

How is Temporal (giant cell) arteritis treated, when, and why?

A

Treat with high-dose corticosteroids prior to temporal artery biopsy to prevent vision loss

26
Q

What patient population does Takayasu arteritis affect?

A

Asian females < 40 years old

27
Q

What are 7 signs/symptoms that characterize the presentation of Takayasu arteritis?

A

(1) “Pulseless disease” (weak upper extremity pulses), (2) fever, (3) night sweats, (4) arthritis, (5) myalgias, (6) skin nodules, (7) ocular disturbances

28
Q

What are 2 histologic/lab findings that characterize Takayasu arteritis?

A

(1) Granulomatous thickening and narrowing of aortic arch and proximal great vessels (2) High ESR

29
Q

How is Takayasu arteritis treated?

A

Treat with corticosteroids

30
Q

What are 3 examples of medium-vessel vasculitis?

A

(1) Polyarteritis nodosa (2) Kawasaki disease (3) Buerger disease (thromboangiitis obliterans)

31
Q

What patient population does polyarteritis nodosa affect?

A

Young adults

32
Q

What kind of seropositivity is associated with polyarteritis nodosa, and what percentage of polyarteritis nodosa patients have it?

A

Hepatitis B seropositivity in 30% of patients

33
Q

What are 10 signs/symptoms that characterize the presentation of polyarteritis nodosa?

A

(1) Fever, (2) Weight loss, (3) Malaise, (4) Headache. GI: (5) Abdominal pain, (6) Melena. (7) Hypertension, (8) Neurologic dysfunction, (9) Cutaneous eruptions, (10) Renal damage.

34
Q

What vessels does polyarteritis nodosa typically involve versus not involve?

A

Typically involves renal and visceral vessels, not pulmonary arteries

35
Q

What is the underlying mechanism of polyarteritis nodosa?

A

Immune complex mediated

36
Q

What are 2 histologic/imaging findings associated with Polyarteritis nodosa?

A

(1) Transmural inflammation of the arterial wall with fibrinoid necrosis (2) Innumerable microaneurysms and spasm on arteriogram

37
Q

How is Polyarteritis nodosa treated?

A

Treat with corticosteroids, cyclophosphamide

38
Q

What patient population does Kawasaki disease affect?

A

Asian children < 4 years old

39
Q

What 6 signs/symptoms characterize the presentation of Kawasaki disease?

A

(1) Fever, (2) Cervical lymphadenitis, (3) Conjunctival injection, (4) Changes in lips/oral mucosa (“strawberry tongue”), (5) Hand-foot erythema, (6) Desquamating rash

40
Q

What cardiac complication(s) may arise from Kawasaki disease?

A

May develop coronary artery aneurysms, thrombosis => MI, rupture.

41
Q

How is Kawasaki disease treated?

A

Treat with IV immunoglobulins and aspirin

42
Q

What is another name for Buerger disease? What patient population does it affect?

A

Buerger disease (thromboangiitis obliterans); Heavy smokers, males < 40 years old

43
Q

What are 3 main sign/symptoms/consequences of Buerger disease (thromboangiitis obliterans)? What is the underlying pathophysiology leading to these? What other condition is often present?

A

Intermittent claudication may lead to (1) gangrene, (2) autoamputation of digits, (3) superficial nodular phlebitis; Raynaud phenomenon is often present

44
Q

Besides it being a medium-vessel vasculitis, what else characterizes the kind of vasculitis that occurs in Buerger disease?

A

Segmental thrombosing vasculitis

45
Q

How is Buerger disease (thromboangiitis obliterans) treated?

A

Treat with smoking cessation

46
Q

What are 4 examples of small-vessel vasculitis?

A

(1) Granulomatosis with polyangitis (Wegener) (2) Microscopic polyangitis (3) Churg-Strauss syndrome (4) Henoch-Schonlein purpura

47
Q

What is another name for Granulomatosis with polyangitis?

A

Granulomatosis with polyangitis (Wegener)

48
Q

What are 3 broad areas affected by Granulomatosis with polyangitis (Wegener)? What are the 9 signs/symptoms spread across these broad areas that characterize the presentation of Wegener?

A

UPPER RESPIRATORY TRACT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis; LOWER RESPIRATORY TRACT: hemoptysis, cough, dyspnea; RENAL: hematuria, red blood cell casts

49
Q

What is the triad that characterizes Granulomatosis with polyangitis (Wegener)?

A

Triad: (1) Focal necrotizing vasculitis (2) Necrotizing granulomas in the lung and upper airway (3) Necrotizing glomerulonephritis

50
Q

What are 2 histologic/imaging findings that characterize Granulomatosis with polyangitis (Wegener)?

A

(1) PR3-ANCA/c-ANCA (anti-proteinase (2) CXR: large nodular densities

51
Q

How is Granulomatosis with polyangitis (Wegener) treated?

A

Treat with cyclophosphamide, corticosteroids

52
Q

What is microscopic polyangitis? What 3 organs does it commonly involve, and how so?

A

Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura

53
Q

To what other vasculitis does microscopic polyangiitis present similarly? What distinguishes it from this other vasculitis?

A

Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement. Also, No granulomas.

54
Q

What histologic finding characterizes microscopic polyangiitis?

A

MPO-ANCA/p-ANCA (anti-myeloperoxidase)

55
Q

How is microscopic polyangiitis treated?

A

Treat with cyclophosphamide and corticosteroids

56
Q

What are 4 major signs/symptoms of Churg-Strauss syndrome? What are 3 other organs that it can also involve?

A

Asthma, Sinusitis, Palpable purpura, Peripheral neuropathy (e.g., wrist/foot drop); Can also involve heart, GI, kidneys (pauci-immune glomerulonephritis).

57
Q

How does Churg-Strauss syndrome impact the kidneys?

A

Kidneys (pauci-immune glomerulonephritis)

58
Q

What are 3 histologic/lab findings that characterize Churg-Strauss syndrome?

A

(1) Granulomatous, necrotizing vasculitis with eosinophilia (2) MPO-ANCA/p-ANC (3) High IgE level

59
Q

What is the most common childhood systemic vasculitis?

A

Henoch-Schonlein purpura

60
Q

What condition does Henoch-Schonlein purpura often follow?

A

Often follows URI

61
Q

What is the classic triad of Henoch-Schonlein purpura?

A

Classic triad: (1) Skin: palpable purpura on buttocks/legs (2) Arthralgias (3) GI: abdominal pain, melena, multiple lesions of same age

62
Q

To what is the vasculitis in Henoch-Schonlein purpura secondary? With what condition is HSP associated?

A

Vasculitis secondary to IgA complex deposition; Associated with IgA nephropathy