Ch 7 Pathoma Vascular Path Flashcards
____ 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
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
Arterial wall is comprised of these 3 layers
1) endothelial intimate
2) smooth muscle media
3) connective tissue adventitia
Name 2 clinical features of vasculitis
1) nonspecific symptoms of inflammation (fever, fatigue, weight loss, myalgias); 2) symptoms of organ ischemia (due to luminal narrowing or thrombosis of inflammed vessels)
In vasculitis, you can get symptoms of organ ischemia due to these 2 reasons
luminal narrowing or thrombosis of inflamed vessels (inflamed wall - healing - fibrosis - narrowing OR endothel damage - collagen exposed - clot formation - thrombus)
Name 2 types of large cell vasculitis and which vessels they classically involve
1) temporal (giant cell) arteritis (branches of carotid artery)
2) takayasu arteritis (aortic arch at branch points)
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
giant; granulomatous; carotid; older adults; greater than 50; females; corticosteroids; blindness
Temporal (giant cell) arteritis presents with these 3 main symptoms (and why). This extra symptom is often present. And ___ is often elevated)
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
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.
inflamed; giant cells; intimal fibrosis; segmental; long; does not
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
giant cell; both; takayasu (young asian females); takayasu; both
Takayasu arteritis is large vessel ___ vasculitis that classically involves the ___ at branch points. Presents in ___ (age ___), and classically in ____. Treatment is ___.
granulomatous; aortic arch; adults less than 50; young asian females; corticosteroids
Takayasu arteritis presents with __ and ___ symptoms with a weak or absent __ in the upper extremity (‘____’ disease). ___ is elevated
visual; neurologic; pulse; pulseless; ESR
Name 3 types of medium vessel vasculitis
muscular arteries that supply organs - 1) polyarteritis nodosa; 2) kawasaki disease; 3) buerger disease
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 ___.
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
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
transmural; fibrinoid necrosis; fibrosis; string-of-pearls; corticosteroids; cyclophosphamide
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
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)
Fibrinoid necrosis can be seen in these two cases
vasculitis and malignant hypertension
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.
Reye syndrome (rapidly progressive encephalopathy); kawasaki disease
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 ____.
necrotizing; digits; ulceration, gangrene, autoamputation; Raynaud phenomenon; heavy smoking; smoking cessation
Name 4 types of small vessel vasculitis
arterioles, capillaries, venules - 1) Wegener granulomatosis; 2) Microscopic polyangiitis; 3) Churg-Strauss syndrome; 4) Henoch-Schonlein purpura
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.
necrotizing granulomatous; nasopharynx, lungs, kidneys; serum c-ANCA; necrotizing granulomas; necrotizing vasculitis; cyclophosphamide and steroids; common
“C” disease (wecener)
Wegener granulomatosis classically presents in a ___ female/male with these 3 symptoms.
middle-aged; male; 1) sinusitis or nasopharyngeal ulceration; 2) hemoptysis with bilateral nodular lung infiltrates; 3) hematuria due to rapidly progressive glomerulonephritis
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.
vasculitis; multiple organs; lung and kidney; nasopharyngeal invlvmt; granulomas; p-ANCA; cyclophosphamide; corticosteroids; common
Churg-Strauss syndrome is a small vessel ___ ___ inflammation with ____ involving ____, especially ___ and ___. ___ and __ are often present. ___ levels correlate with disease activity
necrotizing granulomatous; eosinophils; multiple organs; lungs; heart; asthma; peripheral eosinophilia; serum p-ANCA
Of the small vessel vasculitis, which is/are granulomatous? p-ANCA? c-ANCA? involves lungs? eosinophils? IgA complex deposition
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
What is the most common vasculitis in children?
Henoch-Schonlein purpura
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.
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
____ 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.
Hypertension; systemic; pulmonary; HTN; 140/90; 120/80; primary; secondary
Primary HTN is due to ___ (95% of all HTN cases). Name 6 risk factors.
unknown etiology; age, race (african americans have increased risk, asians have decreased), obesity, stress, lack of physical activity, high salt diet
Secondary HTN (5%) is due to an identifiable etiology. ___ is a common cause.
renal artery stenosis (renovascular hypertension)
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.
decreased; juxtaglomerular apparatus (JGA); renin; angiotensinogen; angiotensin I; angiotensin II; angiotensin converting enzyme (ACE); rise
Angiotensin II raises blood pressure in these 2 ways.
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)
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.
glomerulosa; cortex; increases; distal convoluted tubule; expanding
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
renin; unilateral atrophy of the affected kidney (due to low blood flow)
Name 2 important causes of renal artery stenosis and who you see it in
1) atherosclerosis (elderly males)
2) fibromuscular dysplasia (young females)
Fibromuscular dysplasia is a ___ defect of the ___, resulting in irregular thickening of __ and __ sized ___, especially the ___.
developmental; blood vessel wall; large; medium; arteries; renal artery
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.
mild; moderate; benign; clinically silent; vessel; organ; slowly (over time)
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
severe; 180/120; 5; preexisting benign HTN; de novo; acute end-organ damage (e.g. acute renal failure, headache and papilledema)
Arteriosclerosis is literally ___ due to ___ of the blood vessel wall. Name the 3 pathologic patterns
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)
Atherosclerosis is a ___ that obstructs blood flow. It consists of a ___ core (mostly ___) with a ___ cap. It often undergoes ___.
intimal plaque; necrotic lipid; cholesterol; fibromuscular; dystrophic calcification
Atherosclerosis involved __ and __ sized arteries. Name the 4 most commonly affected.
large; medium; abdominal aorta; coronary artery; popliteal artery; internal carotid artery
Name 4 modifiable risk factors for atherosclerosis
1) hypertension; 2) hypercholesterolemia (LDL increases risk, HDL decreases); 3) smoking; 4) diabetes
Name 3 non-modifiable risk factors for atherosclerosis
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)
How does gender affect your risk for atherosclerosis?
increased risk in males and postmenopausal females (since estrogen is protective)
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 ___.
endothelium; lipids; intima; oxidized; macrophages; scavenger; foam; extracellular matrix; smooth muscle
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 ____.
fatty streaks; intima; lipid-laden macrophages; early; teenagers; atherosclerotic plaque
Name 4 complications of atherosclerotic plaques
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)
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?
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%
A major complication of atherosclerosis occurs when plaque ruptures leading to ___, which can result in ___ (coronary artery) and ____ (middle cerebral artery)
thrombosis; MI; stroke
Plaque rupture with embolization results in ___, characterized by ___ within the embolus
atherosclerotic embolus; cholesterol crystals (looks like cholesterol clefts)
Arteriolosclerosis is narrowing of ___. It is divided in to these 2 types.
small arterioles; hyaline and hyperplastic types
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.
proteins leaking; thickening; proteins; long-standing benign hypertension or diabetes
Hyaline arteriolosclerosis results in reduced ___ with end-organ ___. Classically it produces ___ that slowly progresses to ____.
vessel caliber; ischemia; glomerular scarring (arteriolonephrosclerosis); chronic renal failure
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 ___.
thickening; smooth muscle; onion-skin; malignant hypertension; vessel caliber; ischemia
Hyperplastic arteriolosclerosis may lead to ___ necrosis of the vessel wall with ___. Classically causes ___ with a characteristic ‘____’ appearance
fibrinoid; hemorrhage; acute renal failure; flea-bitten (pinpoint hemorrhages due to blood vessels being blown out)
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?
hyaline; hyperplastic; both; hyperplastic; hyaline; hyaline
____ 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
Mönckeberg Medial Calcific Sclerosis
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.
intimal; media; proximal; 10; pre-existing
What is the most common cause of aortic dissection? What else is it associated with? What group presents with each?
hypertension: older adults
inherited defects of connective: tissue younger individuals
Hypertension results in ____ of the vasa vasorum. Decreased flow causes ___ of the media, and can ultimately cause an aortic dissection.
hyaline arteriolosclerosis; atrophy
These 2 inherited connective tissue defects classically lead to weakness of the connective tissue in the ___ (cystic medial necrosis). What do the defects affect?
media;
Marfan syndrome: defect of fibrillin (what elastic fibers are laid down on)
Ehlers-Danlos syndrome: defective formation of collagen
How does aortic dissection present?
Sharp, tearing chest pain that radiates to the back
Name the 3 complications of aortic dissection and the one that is the most common cause of death
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)
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.
balloon; dilation; weakness; tertiary syphilis; endarteritis (inflammation of small vessels); vaso vasorum; luminal; flow; atrophy; tree-bark
What is the major complication of thoracic aneurysm? Name 3 others.
dilation of the aortic valve root resulting in aortic valve insufficiency; 1) compression of mediastinal structures (airway or esophagus); 2) thrombosis; 3) aneurysm
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 ___.
balloon; dilation; renal arteries; aortic bifurcation; atherosclerosis; male; smokers; 60; hypertension
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
diffusion barrier; weakness; atrophy; pulsatile abdominal mass
What is the major complication of abdominal aneurysm? And what is the triad presentation? Name 3 other complications
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
Hemangioma is a benign/malignant tumor comprised of ___. Commonly present at ___. Often regresses/grows during childhood. Most often involves ___ and ___.
benign; blood vessels; birth; regresses; skin; liver
Angiosarcoma is a benign/malignant proliferation of ___. It is highly aggressive. Common sites include these 3.
malignant; endothelial cells; skin, breast, liver
Name 3 exposures associated with liver angiosarcoma
1) polyvinyl chloride (PVC); 2) arsenic; 3) Thorotrast
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.
low; malignant; endothelial cells; HHV-8; purple patches, plaques, nodules; visceral
Name the 3 groups Kaposi sarcoma is classically seen in and the treatment for each
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)
