Ch 7 Pathoma Vascular Path

Question 1

____ is inflammation of the blood vessel wall. The etiology is usually ___. Most cases are/are not infectious. Divided into these three types, that comprise these 3 vessels

Answer 1

Vasculitis; unknown; are not; 1) large vessel = aorta and major branches; 2) medium vessel = muscular arteries that supply organs; 3) small = arterioles, capillaries, and venules

Question 2

Arterial wall is comprised of these 3 layers

Answer 2

1) endothelial intimate
2) smooth muscle media
3) connective tissue adventitia

Question 3

Name 2 clinical features of vasculitis

Answer 3

1) nonspecific symptoms of inflammation (fever, fatigue, weight loss, myalgias); 2) symptoms of organ ischemia (due to luminal narrowing or thrombosis of inflammed vessels)

Question 4

In vasculitis, you can get symptoms of organ ischemia due to these 2 reasons

Answer 4

luminal narrowing or thrombosis of inflamed vessels (inflamed wall - healing - fibrosis - narrowing OR endothel damage - collagen exposed - clot formation - thrombus)

Question 5

Name 2 types of large cell vasculitis and which vessels they classically involve

Answer 5

1) temporal (giant cell) arteritis (branches of carotid artery)
2) takayasu arteritis (aortic arch at branch points)

Question 6

Temporal (___ cell) arteritis is a large vessel ___ vasculitis that classically involves branches of the ____. It is the most common form of vasculitis in ___ (age __), and usually affects females/males. Treatment is ___, and there is a high risk of __ w/o tx

Answer 6

giant; granulomatous; carotid; older adults; greater than 50; females; corticosteroids; blindness

Question 7

Temporal (giant cell) arteritis presents with these 3 main symptoms (and why). This extra symptom is often present. And ___ is often elevated)

Answer 7

1) headache (temporal artery invlvmt); 2) visual disturbances (ophthalmic artery invlvmt); 3) jaw claudication (pain with chewing); 4) flu like symptoms with joint and muscle pain (polymalgia rheumatica); ESR

Question 8

A biopsy of temporal giant cell arteritis reveals ___ vessel wall with __ and ___. Lesions are ___, so diagnosis requires bx of a long/short segment and a negative bx does/does not exclude disease.

Answer 8

inflamed; giant cells; intimal fibrosis; segmental; long; does not

Question 9

Takayasu arteritis vs temporal giant cell arteritis: which one presents in adults over 50, which one presents in females, which one presents in asians, which one presents at the aortic arch branch points, which ones is granulomatous

Answer 9

giant cell; both; takayasu (young asian females); takayasu; both

Question 10

Takayasu arteritis is large vessel ___ vasculitis that classically involves the ___ at branch points. Presents in ___ (age ___), and classically in ____. Treatment is ___.

Answer 10

granulomatous; aortic arch; adults less than 50; young asian females; corticosteroids

Question 11

Takayasu arteritis presents with __ and ___ symptoms with a weak or absent __ in the upper extremity (‘____’ disease). ___ is elevated

Answer 11

visual; neurologic; pulse; pulseless; ESR

Question 12

Name 3 types of medium vessel vasculitis

Answer 12

muscular arteries that supply organs - 1) polyarteritis nodosa; 2) kawasaki disease; 3) buerger disease

Question 13

Polyarteritis nodosa is a medium vessel ___ vasculitis involving multiple ___, but sparing the ___. Classically presents in old/young adults with these 4 symptoms. It is associated with ___.

Answer 13

necrotizing; organs; lungs; young; 1) hypertension (renal artery invlvmt); 2) abdominal pain with melena (mesenteric artery invlvmt); 3) neurologic disturbances; 4) skin lesions; serum HBsAg

Question 14

Polyarteritis nodosa involves lesions of varying stages. Early lesions consist of ___ inflammation with ___. It eventually heals with ___ producing a ‘____’ appearance on imaging. Tx is ___ and ___, and it is fatal if not treated

Answer 14

transmural; fibrinoid necrosis; fibrosis; string-of-pearls; corticosteroids; cyclophosphamide

Question 15

Kawasaki disease is a medium cell vasculitis that classically affects ___ (___ age). Presents with ___ signs (including these 4). ___ artery involvement is common, which leads to risk for these two things. Tx is __ and __. Disease is self limited

Answer 15

Asian children; less than 4 years old; nonspecific; fever, conjunctivitis, erythematous rash of palms and soles, enlarged cervical lymph nodes; coronary; thrombosis with MI; aneurysm with rupture; aspirin and IVIG
(kawasaki motorcycle)

Question 16

Fibrinoid necrosis can be seen in these two cases

Answer 16

vasculitis and malignant hypertension

Question 17

Usually you don’t give aspirin to a child with a viral illness for risk of developing ___, but you do give aspirin to children with this disease.

Answer 17

Reye syndrome (rapidly progressive encephalopathy); kawasaki disease

Question 18

Buerger’s disease is medium vessel ___ vasculitis involving the ___. It presents with ___, ___, and ___ of fingers and toes. ___ is often present. It is highly associated with ____, and tx is ____.

Answer 18

necrotizing; digits; ulceration, gangrene, autoamputation; Raynaud phenomenon; heavy smoking; smoking cessation

Question 19

Name 4 types of small vessel vasculitis

Answer 19

arterioles, capillaries, venules - 1) Wegener granulomatosis; 2) Microscopic polyangiitis; 3) Churg-Strauss syndrome; 4) Henoch-Schonlein purpura

Question 20

Wegener granulomatosis is a small vessel ___ ___ vasculitis involving these 3 locations. ____ levels correlate with disease activity. Bx reveals large ___ ____ with adjacent ___ ___. Treatment is ___ and ___. Relapses are common/rare.

Answer 20

necrotizing granulomatous; nasopharynx, lungs, kidneys; serum c-ANCA; necrotizing granulomas; necrotizing vasculitis; cyclophosphamide and steroids; common

“C” disease (wecener)

Question 21

Wegener granulomatosis classically presents in a ___ female/male with these 3 symptoms.

Answer 21

middle-aged; male; 1) sinusitis or nasopharyngeal ulceration; 2) hemoptysis with bilateral nodular lung infiltrates; 3) hematuria due to rapidly progressive glomerulonephritis

Question 22

Microscopic polyangiitis is a small vessel ___ vasculitis involving ____, especially __ and ___. Presentation is similar to Wegener granulomatosis except ___ and ___ are absent. ___ levels correlate with disease activity. Tx is ___ and ___. Relapses are common/rare.

Answer 22

vasculitis; multiple organs; lung and kidney; nasopharyngeal invlvmt; granulomas; p-ANCA; cyclophosphamide; corticosteroids; common

Question 23

Churg-Strauss syndrome is a small vessel ___ ___ inflammation with ____ involving ____, especially ___ and ___. ___ and __ are often present. ___ levels correlate with disease activity

Answer 23

necrotizing granulomatous; eosinophils; multiple organs; lungs; heart; asthma; peripheral eosinophilia; serum p-ANCA

Question 24

Of the small vessel vasculitis, which is/are granulomatous? p-ANCA? c-ANCA? involves lungs? eosinophils? IgA complex deposition

Answer 24

granulomatous: wegener and churg-strauss; p-anca: microscopic polyangiitis and churg-strauss; c-anca: wegeners; lungs: WG, MP, and CSS (these three are also all necrotizing); eosinophils: CSS; IgA: hence-shonelein purpura

Question 25

What is the most common vasculitis in children?

Answer 25

Henoch-Schonlein purpura

Question 26

Henoch-Schonlein Purpura is small vessel vasculitis due to ____. It presents with these 3 symptoms, and usually occurs following ____. Disease is self-limited, but may recur. Treated with ___ if severe.

Answer 26

IgA immune complex deposition; 1) palpable purpura on buttocks and legs; 2) GI pain and bleeding; 3) hematuria (IgA nephropathy); upper respiratory tract infection; steroids

Question 27

____ is increased blood pressure. May involve __ or __ circulation. Systemic ___ is defined as pressure of greater than or equal to ___ mmHg (normal is less than ___mmHg). Can be divided into ___ or __ based on etiology.

Answer 27

Hypertension; systemic; pulmonary; HTN; 140/90; 120/80; primary; secondary

Question 28

Primary HTN is due to ___ (95% of all HTN cases). Name 6 risk factors.

Answer 28

unknown etiology; age, race (african americans have increased risk, asians have decreased), obesity, stress, lack of physical activity, high salt diet

Question 29

Secondary HTN (5%) is due to an identifiable etiology. ___ is a common cause.

Answer 29

renal artery stenosis (renovascular hypertension)

Question 30

Renal artery stenosis causes a increased/decreased blood flow to the glomerulus. The ___ responds by secreting ___, which converts ___ to ____. Angiotensin I is converted to ___, by ____, causing a rise/drop in blood pressure.

Answer 30

decreased; juxtaglomerular apparatus (JGA); renin; angiotensinogen; angiotensin I; angiotensin II; angiotensin converting enzyme (ACE); rise

Question 31

Angiotensin II raises blood pressure in these 2 ways.

Answer 31

1) contracting arteriolar smooth muscle, increasing total peripheral resistance
2) promoting adrenal release of aldosterone, which increases resorption of sodium in the distal convoluted table (expanding plasma volume)

Question 32

Aldosterone is released by the zone ___ of the adrenal ___, and increases/decreases resorption of sodium in the ___ of the kidney, resulting in expanding/contracting plasma volume.

Answer 32

glomerulosa; cortex; increases; distal convoluted tubule; expanding

Question 33

Secondary HTN due to renal artery stenosis causes HTN with increased plasma ___ and bilateral/unilateral atrophy of the kidney(s). Neither feature is seen in primary HTN

Answer 33

renin; unilateral atrophy of the affected kidney (due to low blood flow)

Question 34

Name 2 important causes of renal artery stenosis and who you see it in

Answer 34

1) atherosclerosis (elderly males)

2) fibromuscular dysplasia (young females)

Question 35

Fibromuscular dysplasia is a ___ defect of the ___, resulting in irregular thickening of __ and __ sized ___, especially the ___.

Answer 35

developmental; blood vessel wall; large; medium; arteries; renal artery

Question 36

Benign HTN is a ___ or ___ elevation in blood pressure. Most cases of HTN are benign/malignant. Benign HTN is ___, and it causes ___ and ___ damage quickly/slowly.

Answer 36

mild; moderate; benign; clinically silent; vessel; organ; slowly (over time)

Question 37

Malignant HTN is ___ elevation in blood pressure (greater than ____), comprises __% of HTN cases. May arise from ___ or ___. Presents with ___ (name 3) and is a medical emergency

Answer 37

severe; 180/120; 5; preexisting benign HTN; de novo; acute end-organ damage (e.g. acute renal failure, headache and papilledema)

Question 38

Arteriosclerosis is literally ___ due to ___ of the blood vessel wall. Name the 3 pathologic patterns

Answer 38

hard arteries; thickening; 1) atheroscerosis (intima of L/M bv’s); 2) arteriolosclerosis (small bv’s hyaline or hyper plastic); 3) Monckeberg medial calcific sclerosis (media) of medium arteries)

Question 39

Atherosclerosis is a ___ that obstructs blood flow. It consists of a ___ core (mostly ___) with a ___ cap. It often undergoes ___.

Answer 39

intimal plaque; necrotic lipid; cholesterol; fibromuscular; dystrophic calcification

Question 40

Atherosclerosis involved __ and __ sized arteries. Name the 4 most commonly affected.

Answer 40

large; medium; abdominal aorta; coronary artery; popliteal artery; internal carotid artery

Question 41

Name 4 modifiable risk factors for atherosclerosis

Answer 41

1) hypertension; 2) hypercholesterolemia (LDL increases risk, HDL decreases); 3) smoking; 4) diabetes

Question 42

Name 3 non-modifiable risk factors for atherosclerosis

Answer 42

1) age (number and severity of lesions increase with age); 2) gender (increased risk in males and postmenopausal women - estrogen is protective); 3) genetics (multi-factorial but family hx is highly predictive of risk)

Question 43

How does gender affect your risk for atherosclerosis?

Answer 43

increased risk in males and postmenopausal females (since estrogen is protective)

Question 44

In atherosclerosis formation, damage to ___ allows __ to leak into the ___. Lipids are ___, and then consumed by ___ via ___ receptors, resulting in ___ cells. Inflammation and healing leads to deposition of ___ and proliferation of ___.

Answer 44

endothelium; lipids; intima; oxidized; macrophages; scavenger; foam; extracellular matrix; smooth muscle

Question 45

Morphologically, early atherosclerosis begins as ___ (flat yellow lesions of the ___ consisting of ___). This begins early/late in life (present in most ___). Then it progresses to ____.

Answer 45

fatty streaks; intima; lipid-laden macrophages; early; teenagers; atherosclerotic plaque

Question 46

Name 4 complications of atherosclerotic plaques

Answer 46

1) stenosis of medium-sized vessels results in impaired blood flow and ischemia (PVD, angina, ischemic bowel disease); 2) plaque rupture w/thrombosis (MI, stroke); 3) plaque rupture w/embolization; 4) weakening of vessel wall (aneurysm)

Question 47

Atherosclerosis can cause stenosis of medium sized vessels resulting in impaired blood flow and ischemia leading to these 3 symptoms. When do you start to see symptoms?

Answer 47

1) peripheral vascular disease (lower extremity arteries like popliteal); 2) angina (coronary arteries); 3) ischemic bowel disease (mesenteric arteries); when artery is stenosed more than 70%

Question 48

A major complication of atherosclerosis occurs when plaque ruptures leading to ___, which can result in ___ (coronary artery) and ____ (middle cerebral artery)

Answer 48

thrombosis; MI; stroke

Question 49

Plaque rupture with embolization results in ___, characterized by ___ within the embolus

Answer 49

atherosclerotic embolus; cholesterol crystals (looks like cholesterol clefts)

Question 50

Arteriolosclerosis is narrowing of ___. It is divided in to these 2 types.

Answer 50

small arterioles; hyaline and hyperplastic types

Question 51

Hyaline arteriolosclerosis is caused by ___ into the vessel wall, producing vascular thickening/thinning. ____ are seen as pink hyaline on microscopy. It is a consequence of these 2 things.

Answer 51

proteins leaking; thickening; proteins; long-standing benign hypertension or diabetes

Question 52

Hyaline arteriolosclerosis results in reduced ___ with end-organ ___. Classically it produces ___ that slowly progresses to ____.

Answer 52

vessel caliber; ischemia; glomerular scarring (arteriolonephrosclerosis); chronic renal failure

Question 53

Hyperplastic arteriolosclerosis involves thickening/thinning of the vessel wall by hyperplasia of ____ (with a ‘____’ appearance). It is a consequence of ____, and results in reduced ___ with end-organ ___.

Answer 53

thickening; smooth muscle; onion-skin; malignant hypertension; vessel caliber; ischemia

Question 54

Hyperplastic arteriolosclerosis may lead to ___ necrosis of the vessel wall with ___. Classically causes ___ with a characteristic ‘____’ appearance

Answer 54

fibrinoid; hemorrhage; acute renal failure; flea-bitten (pinpoint hemorrhages due to blood vessels being blown out)

Question 55

Which arteriolosclerosis type is a consequence of benign HTN? malignant HTN? causes reduced vessel caliber with end organ ischemia? causes acute renal failure? chronic renal failure? caused by diabetes?

Answer 55

hyaline; hyperplastic; both; hyperplastic; hyaline; hyaline

Question 56

____ is calcification of the media of muscular (medium-sized) arteries. It is non-obstructive and is not clinically significant. Seen as incidental finding on x-ray or mammography

Answer 56

Mönckeberg Medial Calcific Sclerosis

Question 57

Aortic dissection is a __ tear with dissection of blood through the __ of the aortic wall. It occurs in the proximal/distal __cm of the aorta (high stress region) with ___ weakness of the media.

Answer 57

intimal; media; proximal; 10; pre-existing

Question 58

What is the most common cause of aortic dissection? What else is it associated with? What group presents with each?

Answer 58

hypertension: older adults

inherited defects of connective: tissue younger individuals

Question 59

Hypertension results in ____ of the vasa vasorum. Decreased flow causes ___ of the media, and can ultimately cause an aortic dissection.

Answer 59

hyaline arteriolosclerosis; atrophy

Question 60

These 2 inherited connective tissue defects classically lead to weakness of the connective tissue in the ___ (cystic medial necrosis). What do the defects affect?

Answer 60

media;
Marfan syndrome: defect of fibrillin (what elastic fibers are laid down on)
Ehlers-Danlos syndrome: defective formation of collagen

Question 61

How does aortic dissection present?

Answer 61

Sharp, tearing chest pain that radiates to the back

Question 62

Name the 3 complications of aortic dissection and the one that is the most common cause of death

Answer 62

1) pericardial tamponade (most common cause of death); 2) rupture with fatal hemorrhage; 3) obstruction of branching arteries with resultant end-organ ischemia (renal or coronary)

Question 63

Thoracic aneurysm is a ___-like ___ of the thoracic aorta. It is due to ___ in the aortic wall. Classically seen in this disease, which causes ___ of the ___ resulting in ___ narrowing, decreased __, and ___ of the vessel wall. Results in a “___” appearance of the aorta.

Answer 63

balloon; dilation; weakness; tertiary syphilis; endarteritis (inflammation of small vessels); vaso vasorum; luminal; flow; atrophy; tree-bark

Question 64

What is the major complication of thoracic aneurysm? Name 3 others.

Answer 64

dilation of the aortic valve root resulting in aortic valve insufficiency; 1) compression of mediastinal structures (airway or esophagus); 2) thrombosis; 3) aneurysm

Question 65

Abdominal aortic aneurysm is a __-like __ of the abdominal aorta. Usually arises below ___ but above __. It is primarily due to ___, and is classically seen in male/female ___ (greater than __ years old) with ___.

Answer 65

balloon; dilation; renal arteries; aortic bifurcation; atherosclerosis; male; smokers; 60; hypertension

Question 66

Atherosclerosis can lead to abdominal aneurysm by increasing the ___ to the media, resulting in __ and __ of the vessel wall. Abdominal aneurysm present as a ___ that grows with time

Answer 66

diffusion barrier; weakness; atrophy; pulsatile abdominal mass

Question 67

What is the major complication of abdominal aneurysm? And what is the triad presentation? Name 3 other complications

Answer 67

rupture (especially when >5 cm in diameter); hypotension (since bleeding), pulsatile abdominal mass, flank pain; 1) compression of local structures (ureter); 2) thrombosis; 3) embolism

Question 68

Hemangioma is a benign/malignant tumor comprised of ___. Commonly present at ___. Often regresses/grows during childhood. Most often involves ___ and ___.

Answer 68

benign; blood vessels; birth; regresses; skin; liver

Question 69

Angiosarcoma is a benign/malignant proliferation of ___. It is highly aggressive. Common sites include these 3.

Answer 69

malignant; endothelial cells; skin, breast, liver

Question 70

Name 3 exposures associated with liver angiosarcoma

Answer 70

1) polyvinyl chloride (PVC); 2) arsenic; 3) Thorotrast

Question 71

Kaposi sarcoma is a high/low grade benign/malignant proliferation of ___. It is associated with ___. Presents as ___, ___, and __ on the skin. May also involve __ organs.

Answer 71

low; malignant; endothelial cells; HHV-8; purple patches, plaques, nodules; visceral

Question 72

Name the 3 groups Kaposi sarcoma is classically seen in and the treatment for each

Answer 72

1) older eastern european males (tumor remains localiized to skin - tx surgical removal)
2) AIDS (tumor spreads early - tx anti-retroviral agents to boost immune system)
3) transplant recipients (tumor spreads early - tx involves decreasing immunosuppression)