L31- CVS Pathology VIII

Question 1

Microscopic polyangiitis, aka (1), involves the (2) of (3) type vessels, specifically in the (4) layer.

Answer 1

1- hypersensitivity/leukocytoclastic vasculitis
2- necrotizing vasculitis
3- arterioles, capillaries, small venules
4- tunica media: segmental fibrinoid necrosis of media with focal transmural necrotizing lesions via PMNs

Question 2

Microscopic polyangiitis, aka (1), importantly never exhibits (2) because it only involves (3). It will effect (4) organs at (same/varying) times

Answer 2

1- hypersensitivity/leukocytoclastic vasculitis
2- infarctions
3- small vessels (arterioles, capillaries, small venules)
4- all organs, including lungs
5- same time- lesions are all the same age

Question 3

Microscopic polyangiitis results from a reaction to (1) or (2). Most affected people are positive for (3) in blood and negative for (4).

Answer 3

1- drugs- penicilline
2- infections- Strep
3- MPO-ANCA, p-ANCA (70% cases)
4- IgG pauci-immune

Question 4

list the clinical features of Microscopic Polyangiitis

Answer 4

• hemoptysis (blood in sputum)
• hematuria, proteinuria
• hematochezia
• cutaneous purpura

-responds to removal of offending agent

Question 5

Granulomatosis with polyangiitis, aka (1), usually affect people of (2- age, sex). Most affected people are positive for (3) in blood. It is also considered to be a (4) reaction and or (5) response/reaction.

Answer 5

1- Wegener Granulomatosis
2- 40 y/o, M>F
3- PR3-ANCA, c-ANCA (95%)
4- cell mediated hypersensitivity reaction to inhaled agent, infectious or environmental
5- dramatic response to immuno-suppressive therapy

Question 6

Granulomatosis with polyangiitis, aka (1), has the following classic triad: (2).

Answer 6

Wegener Granulomatosis

1) acute necrotizing granulomas of upper / lower respiratory tract
2) focal necrotizing or granulomatous vasculitis affecting small to medium vessels
3) renal disease in focal or necrotizing form, often crescentic glomerulonephritis (RPGN)

Question 7

list the clinical features of WG (include % chance Sx is evident)

also include the prognosis

Answer 7

(Granulomatosis with polyangiitis / Wegener Granulomatosis)

• persistent pneumonitis with bilateral nodular and cavitary infiltrates (95%) => hemoptysis
• chronic sinusitis (90%), mucosal ulceration of nasopharynx (75%)
• renal disease (or evidence of via hematuria, ARF; 80%)

-80% Pts die w/in 1 yr if untreated

Question 8

WG:
-gross appearance of the upper respiratory tract (include parts) will exhibit (1) and the lungs will show (2)

-histologically various areas will exhibit (3) lesions made up (4) cells that will eventually develop into (5)

Answer 8

1- ulcerative lesions: nose, pharynx, palate
2- dispersed focal necrotizing granulomas –> coalesce into nodules that can undergo cavitations

3- necrosis rimmed by granulomas
4- (granulomas made up of) lymphocytes, plasma cells, macrophages, variable number of giant cells
5- progressive fibrosis and organization

Question 9

Churg-Strauss Syndrome is described as (1) with (2) as apparent symptoms. It is usually positive for (3) in blood. It will be exhibited by (4) in the heart, (5) in the kidneys, and (6) in the skin.

Answer 9

1- allergic granulomatosis and angiitis
2- allergic rhinitis, bronchial asthma, peripheral eosinophilia
3- MPO-ANCA, p-ANCA

4- coronary arteritis and myocarditis (fatal)
5- rarely involved
6- palpable purpura

Question 10

For the following vasculitis, indicate if there asthma, gramulomas, and eosinophilia.

(1) microscopic polyangitis
(2) granulomatosis with polyangitis
(3) Churg-Strauss

Answer 10

1- none

2- just granulomas

3- asthma, granulomas, eosinophilia

Question 11

anuerysms are the result of (1) weakness due to (2) or (3) cause

it is classified based on (4), (5), (6)

Answer 11

1- tunica media weakness
2, 3- congenital, acquired

4- composition of wall (layers involved)
5- gross morphology (shape)
6- etiology

Question 12

describe the types of aneursyms based on: composition of wall

Answer 12

True aneurysm (saccular): involves all 3 BV layers, blood remains in circulatory system // atherosclerotic, syphilitic, congenital aneurysm

False (pseudoaneurysm): extravascular hematoma, communicates with intravascular space

Question 13

describe the types of aneursyms based on: gross morphology / shape

Answer 13

Fusiform: diffuse, circumferential dilations of long vascular segment (more common than saccular)

Saccular: spherical outpouchings involving only one portion of vessel wall- not extended all the way around circumference // usually contains thrombus

Question 14

describe the types of aneursyms based on: etiology

Answer 14

• atherosclerotic (AAA)
• syphilitic (thoracic aorta)
• mycotic (infective)
• vasculitic (PAN, Kawasaki’s)
• congenital (tunica media defect- Marfan’s, berry aneurysm)
• iatrogenic (arteriovenous aneurysms for CRF Pts on dialysis)

Question 15

define iatrogenic aneursyms

Answer 15

an arteriovenous aneursym for CRF pts on dialysis

Question 16

Atherosclerotic aneurysms most affect (1) artery, evident by a (2) change, and mostly affects (3) age group.

Answer 16

1- abdominal aorta (AAA)
2- 50% diameter expansion
3- after 50 y/o (<6% after 80 y/o)

Question 17

atherosclerotic aneurysms are initiated by (1) proteins via (2) cells that start to (3); there is also a deficiency in (4)

Answer 17

(mostly AAAs)
1- MMPs: matrix metalloproteinases
2- macrophages
3- degrade all ECM components: collagen, elastin, proteoglycans, laminin, fibronectin
4- tissue inhibitor of proteinases (MMP)

Question 18

describe the gross appearance of AAAs

Answer 18

(atherosclerotic aneurysms)

• mostly distal to renal arteries and proximal to bifurcation
• fusiform (entire circumference) > saccular
• mostly lined by raised, ulcerated, calcified atherosclerotic lesions

Question 19

describe the microscopic appearance of AAAs

Answer 19

(atherosclerotic aneurysms)

• arterial wall destruction with fibrotic tissue replacement
• thickened and focally inflamed adventitia

Question 20

AAAs usually present with (1) and many times a (2). The complications of AAAs include (3), (4), (5), (6), (7)

Answer 20

1- asymptomatic
2- abdominal mass

3- occlusion of branched vessels: renal, SMA, IMA, vertebral vessels
4- embolism from atheroma / mural thrombus
5- impingement of adjacent structure: ureter compression, vertebral erosion
6- hemorrhage (rupture)
7- salmonella infection (mycotic aneurysm)

Question 21

The biggest risk with AAAs is (1). The risk of (1) is based on (2).

Answer 21

1- massive or fatal hemorrhage due to rupture
2- size:
-2% for small AAAs
-25-40% for ones larger than 6 cm

Question 22

how are AAAs treated

Answer 22

-if large enough: surgical replacement by prosthetic grafts

Note operative mortality differences:

• 5% in unruptured AAA
• > 50% in ruptured AAA (emergency Sx)

Question 23

Syphilitic aneurysms are complications of (1). It usually affects (2).

There is a inflammatory response to (3) which results in (4) of (2). (2) will have a (5) as a result causing (6) and (7).

Answer 23

1- tertiary syphilis
2- thoracic aorta
3- spirochetes
4- obliterative endarteritis of vasa vasorum (small BVs that supply vessel layers) of aorta
5- lumen narrowing
6- ischemic injury to tunica media of aorta
7- medial destruction and medial weakening –> followed by chronic inflammation and scarring

Question 24

describe the appearance of syphilitic aneurysms

Answer 24

(thoracic aorta)

• fibrosis of vascular wall —- ‘tree bark appearance’
• aortic ‘intima wrinkling’ due to secondary atherosclerosis –> narrows or occludes coronary ostea
• aortic ring dilation –> valvular insufficiency –> massive LV hypertrophy (= cor bovinum / ‘cow’s heart’)

Question 25

describe the clinical course (including complications) of syphilitic aneurysms

Answer 25

(thoracic aorta)
-encroaches on mediastinal structures: respiratory difficulties, swallowing difficulties, persistent cough (laryngeal nerve compression)

• pain caused by erosion of ribs/vertebrae
• aortic incompetence –> LVH –> CHF (most common cause of death)
• cardiac ischemia due to coronary ostia obstruction
• rupture

Question 26

Mycotic aneurysms result from (1), commonly in (2) arteries. They can originate from (3), (4), (5)

Answer 26

1- vessel wall weakening secondary to microbial infection
2- aorta, cerebral, mesenteric, renal, splenic arteries

3- site of dislodged septic emobli final location (complication of infective endocarditis)
4- extension of adjacent suppurative process, i.e. Tb infection, bacterial abscess
5- circulating organisms –> directly infecting arterial wall

Question 27

A common cause of congenital aneursyms is (1) due to a (2) mutation inherited in (3) fashion.

Mutation of (2) results in the following vascular (4), skeletal (5), and ocular (6) abnormalities. Prognosis of (1) is (7).

Answer 27

1- Marfan Syndrome
2- fibrillin-1 (required for elastin development)
3- AD

4- no elastin –> more incompetent vessels that are less resistant to stress => aneursyms, aortic dissections, valve lesions
5- elongate axial bones (tall, slender body, more in lower body) + long thin extremities/fingers
6- subluxation of lens, (b/c cillary body rich in fibrillin)

7- death in 30s, 40s if untreated

Question 28

(1) are thin-walled aneurysms of the Circle of Willis. They are usually (large/small), (fusiform/saccular), and (present at/develop after) birth. It is often associated with (5).

Answer 28

(often off ACA)
1- berry aneurysm
2- small
3- saccular
4- develop after birth, although congenital attenuation
5- PKD (polycystic kidney disease)

Question 29

Berry aneurysms develop over time because of (1). Rupture of them is caused by (2) and leads to (3) evident by (4) presentation.

Answer 29

1- arterial media is congenitally attenuated
2- (any time) but often during inc intracranial pressure (pooping, organism)
3- subarachnoid hemorrhage
4- severe ‘thunderclap’ HA, coma

Question 30

Aortic dissection, aka (1), is defined as (2) and ruptures will result in (3).

It is commonly evident in (4) people and sometime evident in (5) people.

Answer 30

1- dissecting hematoma
2- blood collection between and along laminar planes of media and its extension along vessel length
3- massive hemorrhage

4- 40-60 y/o HTN Pts (90%)
5- young ppl w/ CT disease (Marfan’s)

Question 31

Aortic dissection can have one of the following causes:….

Answer 31

1) HTN: vasa vasora hypertrophy => lumen narrowing + ECM degeneration –> loss of SMC (due to pressure and ischemia)
2) abnormal CT (Marfan, Ehler-Danlos syndrome)
3) arterial cannulation complication (e.g. during diagnostic catheterization, cardiopulmonary bypass)
4) pregnancy induced: unknown, possibly b/c hormone vascular remodeling, perinatal hemodynamic stress

Question 32

describe the 2 types of aortic dissections

Answer 32

(depends of portion of aorta affected)
Type A / Proximal lesions: more common, ascending aorta, high mortality, needs rapid medical/surgical Tx

Type B / Distal lesions: descending aorta distal to L subclavian; better prognosis, conservative management

Question 33

what are the clinical features of aortic dissection

Answer 33

• sudden onset of ‘tearing’ chest pain radiating to back (between scapula) – moves down as dissection progresses
• loss of one or more pulses
• many other features of complications

Question 34

describe gross appearance of aortic dissection

Answer 34

• intimal tear, transverse, sharp, jagged
• separated inner 2/3 of aorta from outer 1/3
• external rupture => hemorrhage, tamponade
• lumen rupture –> ‘double barrel’ aorta

Question 35

describe histological appearance of aortic dissection

Answer 35

• cystic medial degeneration: focal loss of elastic and muscle fibers in media –> cystic spaces with myxoid material
• NO inflammation

Question 36

list the complications of aortic dissection

Answer 36

-DEATH, most common due to rupture into pericardium, pleura, or peritoneum

-retrograde dissection into aortic root –> disrupted aortic valve and coronary arteries
-cardiac tamponade
-extension into renal, mesenteric, iliac arteries => critical obstruction
-spinal artery compression => transverse myelitis
(-‘double barrel’ aorta: tear at either side of dissection, both on the luminal side => duel blood flow)