Y2S1B2 - Path - Pathoma Vascular

Front 1

What are the 2 main categories of clinical features of vasculitis?

Back 1

Nonspecific symptoms of inflammation

Symptoms of organ ischemia

Front 2

MC vasculitis in adult females, >50 yrs old?

Back 2

Temporal (Giant Cell) Arteritis

Front 3

A 67 yo woman presents w/ headache, visual disturbances, and pain in the jaw/ear while chewing. She also complains of some mild joint and muscle pain and flu-like symptoms.

Biopsy shows giant cells and intimal fibrosis.

a) What does she likely have?
b) Without treatment, what does she have a risk of?

Back 3

a) Temporal (Giant Cell) Arteritis
headache d/t temporal artery
visual d/t ophthalmic artery
jaw claudication d/t blood to muscles
b) Blindness (irreversible, d/t ophthalmic artery involvement)

Front 4

Which vasculitis has segmental lesions and therefore, you must biopsy a long segment of the vessel.

What is important about a negative biopsy?

Back 4

Temporal (Giant Cell) Arteritis

Negative biopsy does NOT exclude disease!

Front 5

Granulomatous vasculitis that usually involves branches of the carotid artery?

Back 5

Temporal (Giant Cell) Arteritis

Front 6

Granulomatous vasculitis that usually involves the aortic arch at branch points?

Back 6

Takayasu Arteritis

Front 7

MC vasculitis in adult Asian females <50 yrs old?

Back 7

Takayasu Arteritis

Front 8

A 32 yo Chinese woman presents w/ visual disturbances and lightheadedness. Upon physical exam, you find that she has a decreased radial pulse.

What does she likely have?

Back 8

a) Takayasu Arteritis
visual + neurologic symptoms
"pulseless dz" - weak or absent pulse in UE

Front 9

The treatment for both Temporal (Giant Cell) Arteritis and Takayasu Arteritis is what?

Back 9

Corticosteroids

Front 10

"pulseless disease"

Back 10

Takayasu Arteritis

Front 11

A 30 yo man presents w/ HTN, abdominal pain, black tar-like stools, lightheadedness, and skin lesions.

Biopsy shows lesions of varying stages with transmural inflammation with fibrinoid necrosis.

What does he likely have?

Back 11

Polyarteritis Nodosa

young adult
HTN (renal artery involvement)
abd pain w/ melena (mesenteric artery involvement)
neurologic disturbances
skin lesions

Front 12

Polyarteritis is a (granulomatous/necrotizing) vasculitis involving multiple organs, but what organ is spared?

Back 12

Necrotizing

Lungs

Front 13

Elevated serum HBsAg is a/w what vasculitis?

Back 13

Polyarteritis Nodosa

Front 14

"string of pearls" appearance

Back 14

Polyarteritis Nodosa

Front 15

Polyarteritis Nodosa, Wegener Granulomatosis and Microscopic Polyangiitis are treated with what?

Back 15

Corticosteroids (steroids) and cyclophosphamide

Front 16

MC vasculitis in Asian children <4 yrs old?

Back 16

Kawasaki disease

Front 17

A Japanese 33 yo child presents with fever, conjunctivitis, enlarged cervical lymph nodes, and an erythematous rash on the palms and soles.

The treatment for this disease is ASPIRIN and IVIG. What disease does this child have?

Back 17

Kawasaki disease!

presents typically in Asian children <4 yrs old
nonspecific signs
disease is SELF-LIMITED

appears kind of like a virus, but aspirin is contraindicated in children with viral infections, because of the risk of Reye's Syndrome (potentially fatal).

Front 18

Coronary artery involvement in Kawasaki disease is common and therefore leads to a risk of what 2 things?

Back 18

1. thrombosis with MI
2. aneurysm with rupture

Front 19

How can you remember that kids with Kawasaki Disease typically present with rashes on their palms and soles?

Back 19

Picture a kid on a Kawasaki motorcycle. Their palms and soles are on the handles and pedals!

Front 20

Necrotizing vasculitis involving the digits?

Back 20

Wegener Granulomatosis

Front 21

What are the C's of Wegener (WeCener) Granulomatosus?

Back 21

C on a stick figure - affects nasopharynx, lungs, and kidneys
c-ANCA
Cyclophosphamide as main tx

Front 22

A 45 yo man presents with sinusitis, hemoptysis, and hematuria. Chest xray shows bilateral nodular infiltrates. Biopsy shows large, necrotizing granulomas w/ adjacent necrotizing vasculitis.

a) What is the likely disease?
b) What do you expect to be elevated in the serum?
c) After treatment, what is a good thing to keep in mind?

Back 22

a) Wegener Granulomatosus
b) c-ANCA
c) Relapses are common!

middle-aged male
sinusitis or nasopharyngeal ulceration
hemoptysis w/ bilateral nodular lung infiltrates
hematuria d/t rapidly progressive glomerulonephritis

Front 23

Buerger disease is highly a/w what?

Back 23

Heavy smoking. Therefore, treatment is smoking cessation

Front 24

How does Buerger disease present?

Back 24

ulceration, gangrene, and autoamputation of fingers and toes, often with Raynaud phenomenon (vasospasm cuts blood supply, color changes from white to blue to red) in a smoker!

Front 25

Necrotizing granulomatous vasculitis involving nasopharynx, lungs and kidneys?

Back 25

Wegener Granulomatosus

Front 26

A 45 yo man presents with hemoptysis and hematuria. Chest xray shows bilateral nodular infiltrates. Biopsy shows absence of granulomas.

a) What is the likely disease?
b) What do you expect to be elevated in the serum?
c) After treatment, what is a good thing to keep in mind?

Back 26

a) Microscopic Polyangitis
b) p-ANCA
c) Relapses are common

Front 27

Main differences b/w Wegener Granulomatosis and Microscopic Polyangitis?

Back 27

Wegener has granulomas and involves nasopharynx, with elevated c-ANCA.
On the other hand, Microscopic Polyangitis does NOT have granulomas NOR does it involve the nasopharynx. It also has elevated p-ANCA instead of elevated c-ANCA.

Front 28

Necrotizing vasculitis involving multiple organs, especially the lung and kidney?

Back 28

Microscopic Polyangitis

Front 29

Necrotizing granulomatous inflammation w/ eosinophils involving multiple organs, especially lungs and heart?

Back 29

Churg-Strauss Syndrome

Front 30

What are the main differences between Microscopic Polyangitis and Churg-Strauss syndrome?

Back 30

Churg-Strauss has granulomas, and often presents with asthma and peripheral eosinophilia.

Front 31

In Churg-Strauss Syndrome, (c-ANCA/p-ANCA) is elevated in the serum.

Back 31

p-ANCA

Front 32

What is the difference between p-ANCA and c-ANCA.

Back 32

p-ANCA is located close to the nucleus (perinuclear)
c-ANCA is located near the periphery of the cell (cytoplasmic)

Front 33

What is the MOST common vasculitis in children?

Back 33

Henoch-Schonlein Purpura

Front 34

A child presents with palpable purpure on the buttocks and legs, GI pain and bleeding, and hematuria. Prior to this, the child suffered from and URI.

a) What is the likely disease?
b) What should be your plan for this child?

Back 34

a) Henoch-Schonlein Purpura

b) It is self-limited but may recur, so don't really need to do much. However, if it is severe, you can treat with steroids.

Front 35

What causes Henoch-Schonlein Purpura?

Back 35

IgA immune complex deposition

(IgA nephropathy is the cause for hematuria; purpura are palpable d/t inflammation)

Front 36

Systolic pressure is due to _______ while diastolic pressure is due to __________.

Back 36

Stroke volume
TPR

Front 37

What is the etiology of primary HTN, which is about 95% of cases of HTN?

Back 37

unknown etiology!

Front 38

In relation to race and HTN, there is an increased risk in _______________ and decreased risk in ___________.

Back 38

African Americans; Asians

Front 39

How does a high-salt diet increase the risk of primary HTN?

Back 39

It increased blood volume and TPR, therefore affecting both systolic and diastolic pressures.

Front 40

Stenosis of what artery is a common cause of secondary HTN?

Back 40

Renal artery (renovascular HTN)

It decreases blood flow to the glomerulus, and the JGA responds by secreting renin (angiotensinogen -> AT I). AT I is converted to AT II by ACE. AT II raises blood pressure via 2 mechanisms (arteriole SM constriction and inc adrenal release of aldosterone, causing increased Na resorption in DCT).

-> Leads to HTN w/ inc plasma renin and unilateral renal atrophy

Front 41

What features are seen in secondary HTN and NOT in primary HTN?

Back 41

increased plasma renin and unilateral atrophy

Front 42

What is the most common cause of renal artery stenosis in elderly males?
In young females?

Back 42

Atherosclerosis

Fibromuscular dysplasia

Front 43

What is fibromuscular dysplasia?

Back 43

a developmental defect of the blood vessel wall, resulting in irregular thickening of large- and medium-sized arteries, especially renal!

Front 44

Most cases of HTN are (benign/malignant).

Back 44

Benign.

mild/mod BP elevation, clinically silent, damage to vessels and organs slowly over time

Front 45

Malignant HTN is a severe rise in BP, >_______ mmHg.

Back 45

>200/120 mm Hg

Front 46

T/F

Malignant HTN always arises from preexisting benign HTN.

Back 46

False!

May arise from preexisting benign HTN or de novo.

Front 47

A patient presents w/ acute renal failure, headache, and papilledema. BP is 210/125 mm Hg.

What's going on?

Back 47

This is a medical emergency! They have malignant HTN, presenting with acute end-organ damage.

Front 48

An intimal plaque consisting of a necrotic lipic core (mostly _________) with a fibromuscular cap is characteristic of what?

Back 48

(cholesterol)
Atherosclerosis

Front 49

What type of arteriosclerosis involves large- and medium-sized arteries?
Which arteries are commonly affected?

Back 49

Atherosclerosis

abdominal aorta, coronary artery, popliteal artery, and internal carotid artery

Front 50

a) Modifiable risk factors for atherosclerosis?

b) Non-modifiable risk factors for atherosclerosis?

Back 50

a) HTN, hypercholesterolemia, smoking, diabetes

b) age, gender, genetics

Front 51

What is thought to be protective from atherosclerosis?

Back 51

Estrogen

Therefore, higher incidence in males and postmenopausal women

Front 52

What begins as "fatty streaks" early in life (teens)?

Back 52

Atherosclerosis

fatty streaks = flat yellow lesions of intima consisting of lipid-laden macrophages. progresses to atherosclerotic plaque

Front 53

Pathogenesis of atherosclerosis:
1. Damage to endothelium allows a)______ to leak into the b)_______.
2. 1a are oxidized and then consumed by ___________ via scavenger receptors, resulting in ___________.
3. Inflammation and healing leads to deposition of ___________ and proliferation of ____________.

Back 53

1. a) lipids b) intima
2. macrophages; foam cells
3. extracellular matrix; smooth muscle

Front 54

What are the 4 main complications of atherosclerosis (accounting for >50% of disease in Western countries)?

Back 54

1. Stenosis of medium-sized vessels (peripheral vascular dz d/t LE arteries, angina d/t coronary, ischemic bowel dz d/t mesenteric)
2. Plaque rupture w/ thrombosis -> MI (coronary) & stroke (middle cerebral)
3. Plaque rupture w/ embolizatioin
4. Aneurysm (d/t weakening of vessel wall; ex: abdominal aorta)

Front 55

What is the hallmark of atherosclerotic emboli?

Back 55

Cholesterol crystals (clefts)

Front 56

Arteriolosclerosis is divided into what 2 types?

Back 56

Hyalina and hyperplastic types

Front 57

Protein leakage into vessel wall (seen as pink hyaline on microscopy), producing vascular thickening is a/w what?

Back 57

Hyaline arteriosclerosis

Front 58

Hyaline arteriolosclerosis is a consequence of what? and classically leads to what?

Back 58

long-standing benign HTN or diabetes

CHRONIC renal failure

Front 59

Hyperplastic arteriolosclerosis is a consequence of what? and classically leads to what?

Back 59

malignant HTN

ACUTE renal failure

Front 60

Glomerular scarring (aka arteriolonephrosclerosis) is classically produced by what?

Back 60

Hyaline arteriolosclerosis

Front 61

Thickening of the vessel wall by hyperplasia of smooth muscle is a/w what?

Back 61

Hyperplastic arteriolosclerosis

Front 62

"onion-skin" appearance a/w what?

Back 62

Hyperplastic arteriolosclerosis

Front 63

Fibrinoid necrosis of vessel wall w/ hemorrhage may occur with what type of arteriosclerosis?

Back 63

Hyperplastic arteriolosclerosis

Front 64

"flea-bitten" appearance a/w what?

Back 64

Hyperplastic arteriolosclerosis

(kidney, pinpoint hemorrhages)

Front 65

Nonobstructive calcification of the media of muscular (medium-sized) arteries is a/w what?

Back 65

Monckeberg Medial Calcific Sclerosis

Front 66

What is the significance of Monckeberg Medial Calcific Sclerosis?

Back 66

It isn't clinically significant, but you can see it as an incidental finding on x-ray or mammography. For example, in mammography, you are looking for calcification as a sign of cancer, but calcifications d/t Monckeberg Medial Calcific Sclerosis may appear in a vascular pattern.

Front 67

Why and where does aortic dissection typically occur?

Back 67

pre-existing weakness of the media, in the proximal 10 cm of the aorta (high stress region)

Front 68

The most common cause of aortic dissection (older adults) is what?
However, it may also occur in younger individuals with what?

Back 68

HTN (d/t hyaline arteriolosclerosis of vaso vasorum -> atrophy of media)

inherited defects of connective tissue (Marfan and Ehlers-Danlos -> cystic medial necrosis)

Front 69

A patient presents with sharp, tearing chest pain that radiates to the back. What should you suspect?

Back 69

Aortic dissection

Front 70

What is the most common cause of death with aortic dissection?

Back 70

Pericardial tamponade

Front 71

What are some possible complications of aortic dissection?

Back 71

pericardial tamponade, rupture w/ fatal hemororhage, obstruction of branching arteries (ex: coronary or renal) w/ resultant end-organ ischemia

think about the different directions the blood might go through the wall

Front 72

Thoracic aneurysm is classically seen in what, and why?

Back 72

Tertiary syphylis. endarteritis of vaso vasorum -> luminal narrowing, dec flow, atrophy of vessel wall

Front 73

"tree bark" appearance

Back 73

aorta in thoracic aneurysm

Front 74

What is the major complication of a thoracic aneurysm?

Back 74

dilation of aortic valve root -> aortic valve sufficiency

Front 75

What is the major complication of an abdominal aortic aneurysm?

Back 75

rupture, especially when >5 cm

TRIAD: hypotension, pulsatile abdominal mass, flank pain

Front 76

Abdominal aortic aneurysms are primarily d/t what?

Back 76

Atherosclerosis. (increases diffusion barrier to media, resulting in atrophy and weakness)

therefore classically seen in male smokers >60 w/ HTN

Front 77

A 70 yo male smoker presents w/ hypotension, pulsatile abdominal mass, and flank pain. He has a history of HTN. What should you suspect?

Back 77

Abdominal aortic aneurysm

Front 78

Which one DOES blanch with pressure: hemangioma or Kaposi sarcoma?

Back 78

Hemangioma

Front 79

Hemangiomas most often affect skin and what else?

Back 79

Liver

Front 80

Malignant, highly aggressive proliferation of endothelial cells in the skin, breast, or liver?

Back 80

Angiosarcoma

Front 81

What is a/w PVC, arsenic, and Thorotrast?

Back 81

Liver angiosarcoma

Front 82

Low-grade malignant proliferation of endothelial cells a/w HHV-8?

Back 82

Kaposi Sarcoma

Front 83

Kaposi sarcoma classically presents in 3 types of people. How do the tumors present in each and how is each treated?

1. Older Eastern European males
2. AIDS
3. Transplant recipients

Back 83

1. Older Eastern European males - localized to skin; surgical removal

2. AIDS - spreads early; antiretroviral agents to boost immune system

3. Transplant recipients - spreads early; decrease immunosuppression

Front 84

How does Kaposi Sarcoma present?

Back 84

purple patches, plaques and nodules on skin; may have visceral involvement