Card - Path (Part 6: Vascular Disorders)

Question 1

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

Answer 1

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

Question 2

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

Answer 2

Most often in the fingers and toes; Affects small vessels

Question 3

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

Answer 3

(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)

Question 4

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

Answer 4

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)

Question 5

Give 9 examples of vascular tumors.

Answer 5

(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

Question 6

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

Answer 6

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

Question 7

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

Answer 7

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

Question 8

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

Answer 8

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

Question 9

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

Answer 9

Cavernous lymphangioma of the neck. Associated with Turner syndrome.

Question 10

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

Answer 10

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

Question 11

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

Answer 11

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

Question 12

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

Answer 12

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

Question 13

With what 2 exposures is angiosarcoma associated?

Answer 13

Associated with radiation therapy and arsenic exposure.

Question 14

Briefly discuss the progression and resection of angiosarcoma.

Answer 14

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

Question 15

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

Answer 15

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

Question 16

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

Answer 16

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

Question 17

With what 2 pathogens is Kaposi sarcoma associated?

Answer 17

Associated with HHV-8 and HIV

Question 18

For what is Kaposi sarcoma frequently mistaken?

Answer 18

Frequently mistaken for bacillary angiomatosis

Question 19

What are the 3 major categories of vasculitis?

Answer 19

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

Question 20

What are 2 types of large-vessel vasculitis?

Answer 20

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

Question 21

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

Answer 21

Temporal (giant cell) arteritis; Generally elderly females

Question 22

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?

Answer 22

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

Question 23

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

Answer 23

Most commonly affects branches of carotid artery

Question 24

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

Answer 24

(1) Focal granulomatous inflammation (2) High ESR

Question 25

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

Answer 25

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

Question 26

What patient population does Takayasu arteritis affect?

Answer 26

Asian females < 40 years old

Question 27

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

Answer 27

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

Question 28

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

Answer 28

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

Question 29

How is Takayasu arteritis treated?

Answer 29

Treat with corticosteroids

Question 30

What are 3 examples of medium-vessel vasculitis?

Answer 30

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

Question 31

What patient population does polyarteritis nodosa affect?

Answer 31

Young adults

Question 32

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

Answer 32

Hepatitis B seropositivity in 30% of patients

Question 33

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

Answer 33

(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.

Question 34

What vessels does polyarteritis nodosa typically involve versus not involve?

Answer 34

Typically involves renal and visceral vessels, not pulmonary arteries

Question 35

What is the underlying mechanism of polyarteritis nodosa?

Answer 35

Immune complex mediated

Question 36

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

Answer 36

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

Question 37

How is Polyarteritis nodosa treated?

Answer 37

Treat with corticosteroids, cyclophosphamide

Question 38

What patient population does Kawasaki disease affect?

Answer 38

Asian children < 4 years old

Question 39

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

Answer 39

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

Question 40

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

Answer 40

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

Question 41

How is Kawasaki disease treated?

Answer 41

Treat with IV immunoglobulins and aspirin

Question 42

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

Answer 42

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

Question 43

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?

Answer 43

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

Question 44

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

Answer 44

Segmental thrombosing vasculitis

Question 45

How is Buerger disease (thromboangiitis obliterans) treated?

Answer 45

Treat with smoking cessation

Question 46

What are 4 examples of small-vessel vasculitis?

Answer 46

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

Question 47

What is another name for Granulomatosis with polyangitis?

Answer 47

Granulomatosis with polyangitis (Wegener)

Question 48

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?

Answer 48

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

Question 49

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

Answer 49

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

Question 50

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

Answer 50

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

Question 51

How is Granulomatosis with polyangitis (Wegener) treated?

Answer 51

Treat with cyclophosphamide, corticosteroids

Question 52

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

Answer 52

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

Question 53

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

Answer 53

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

Question 54

What histologic finding characterizes microscopic polyangiitis?

Answer 54

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

Question 55

How is microscopic polyangiitis treated?

Answer 55

Treat with cyclophosphamide and corticosteroids

Question 56

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

Answer 56

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

Question 57

How does Churg-Strauss syndrome impact the kidneys?

Answer 57

Kidneys (pauci-immune glomerulonephritis)

Question 58

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

Answer 58

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

Question 59

What is the most common childhood systemic vasculitis?

Answer 59

Henoch-Schonlein purpura

Question 60

What condition does Henoch-Schonlein purpura often follow?

Answer 60

Often follows URI

Question 61

What is the classic triad of Henoch-Schonlein purpura?

Answer 61

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

Question 62

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

Answer 62

Vasculitis secondary to IgA complex deposition; Associated with IgA nephropathy