Blood Vessel Path

Question 1

Focal irregular thickening in Medium and Large Muscular arteries (Carotid, Renal, Splanchnic, Vertebral) due to segmental intimacy and medial Hyperplasia leading to Luminal stenosis. May have the appearance of “Beads on a String.” More common seen in young Women.

Answer 1

Fibromuscular Dysplasia

Question 2

What is the most frequent cause of clinically relevant Sub-Arachnoid Hemorrhage?

Answer 2

Rupture of Saccular (Berry) Aneurysms present in the Circle of Willis

Question 3

Where do 90% Saccular (Berry) Aneurysms typically form?

Answer 3

90% of Saccular Aneurysm in the Head typically form in the Anterior Circulation of the Circle of Willis

Question 4

Aneurysms above what size have a 50% chance of bleeding every year?

Answer 4

Aneurysms greater than 10mm have a 50% chance of bleeding every year

Question 5

What may trigger the rupture of a Saccular (Berry) Aneurysm in the Circle of Willis?

Answer 5

1/3 of Saccular Aneurysms rupture due to increased intracranial pressure. May be due to straining, sexual orgasm, etc.

Question 6

What percentage of people die with their first Saccular (Berry) Aneurysm rupture in the head?

Answer 6

25-50% of people will die with their first Saccular Aneurysm rupture in the head

Question 7

What characterizes a “Giant” Aneurysm

Answer 7

A Giant Aneurysm is greater than 2.5cm and has a dramatically increased risk of rupture

Question 8

Fibromuscular Dysplasia has higher incidence in young women, but is it associated with Oral Contraceptive/Estrogen use? What about genetic inheritance?

Answer 8

Fibromuscular Dysplasia is NOT associated with Oral Contraceptive/Estrogen use. It does however hav some genetic component as there is increased incidence in first degree relatives

Question 9

What is the most commonly affect site of Fibromuscular Dysplasia? What about the second most common? What affect will it have there?

Answer 9

Renal Arteries are the most common

Internal Carotid Arteries are the second most common

Fibromuscular Dysplasia will result in HTN at whatever site it affects

Question 10

What is the stereotypic response of a vessel wall to ANY form of insult/injury?

Answer 10

Intimate Thickening is the stereotypic response of a vessel wall to ANY form of insult/injury

Question 11

At what percent of vessel luminal occlusion are you said to have reached “Critical Stenosis”?

Answer 11

Critical Stenosis is at 70% Occlusion

Question 12

What Cytokines and inflammatory mediators are implicated in the pathogenesis of Atherosclerosis?

Answer 12

Cholesterol Crystals are recognized by the Inflammasome -> IL-1

PDGF, FGF< and TGF-a are involved in the smooth muscle processes of Atherosclerosis

Question 13

What is the difference between a Stable Atherosclerotic Plaque versus a Vulnerable Atherosclerotic Plaque?

Answer 13

A Vulnerable Atherosclerotic plaque has had its overlying fibrous cap weakened due to the ongoing inflammatory processes induce by Atherosclerosis, which causes an increase in ECM degradation compared to ECM synthesis/deposition

Question 14

What Cardiovascular issues might one experience if they have Tertiary or Late Stage Syphilis?

Answer 14

Tertiary Syphilis -> Obliterative Endarteritis (typically of Vasa Vasorum of Thoracic Aorta) -> Ischemia of the Outer Thoracic Aorta Media -> Aneurysm and may even involved Aortic Valve Annulus -> Aortic Regurgitation & Cardiac Tamponade

Question 15

What is Cystic Medial Degeneration?

Answer 15

Cystic Medial Degeneration is the final result of a number of pathological processes which is characterized by disrupted and disorganized elastin filaments and increased ground substance (Proteoglycan)

Question 16

Where do AAA’s typically occur? What are some of the risk factors for AAA?

Answer 16

AAA’s typically occur below the Renal Arteries

Risk factors include Men, Smokers, and 6th decade of life

Question 17

Abdominal Aortic Aneurysm rupture risk is related to size, as such Aneurysms larger than what size are normally surgically managed?

Answer 17

Abdominal Aneurysms greater than 5cm are normally surgically managed as aneurysms greater than 5-6cm can have around a 25% chance of rupture if not greater

Question 18

What is the inheritance of Marfan Syndrome, what gene is involved, what molecule is involved? What Cytokine is heavily involved in the pathogenesis of Marfan’s?

Answer 18

Marfan Syndrome is inherited Autosomal Dominantly, it affects the FBN-1 Gene, and results in defective Fibrillin synthesis

Marfan Syndrome leads to aberrant TGF-Beta Activity which weakens elastic tissue

Question 19

What is the primary risk factor for Aortic Dissection? Besides the primary risk factor, what other risk factors are there?

Answer 19

Hypertension is the primary Risk factor for Aortic Dissection

Being a Male ages 40-60, or having Marfan Syndrome are also risk factors

Question 20

What is the classical clinical presentation of Aortic Dissection?

Answer 20

Sudden Onset severe chest pain, often beginning in the anterior chest, radiating to the back between the scapulae, and moving downward as it progresses

Question 21

What is the connection between Cystic Medial Degeneration and Aortic Dissection?

Answer 21

Cystic Medial Degeneration is what typically allows the Dissection to propagate further through the wall of the vessel

Question 22

Where do most Aortic Dissections arise?

Answer 22

Most Aortic Dissections arise in the Ascennding Aorta, usually within 10cm of the Aortic Valve as this is the area of highest pressure

Question 23

Giant Cell (Temporal) Arthritis/Aortitis:
Sx:
Pathogenesis:

Answer 23

Sx: Headache, Facial Pain (especially over the course of the Superficial Temporal A.), Consitutional Symptoms (fever, fatigue, weight loss), Opthalmic A. Involvement may lead to Diplopia or even Acute vision loss

Pathogenesis: Non-Infectious Vasculitis. CD4+ T Cell Mediated, fragmentation of elastic lamina, intimacy thickening, Multinucleated Giant Cells

Question 24

What is the mots common Vasculitis in Older Patients?

Answer 24

Giant Cell (Temporal) Arteritis/Aortitis is the most common Vasculitis in Older Patients

Question 25

Takayasu Arteritis (Pulseless Disease)
Sx:
Pathogenesis:

Answer 25

Sx: characterized by ocular disturbances and marked weakening of pulses in the UE, pulmonary HTN, retinal hemorrhages, neuro deficits

Pathogenesis: Non-Infectious Vasculitis. Similar histologic all findings as Giant Cell Arteritis - giant cells, intimacy thickening, luminal narrowing, EXCEPT:

• Younger Age Group
• Pulmonary A. Involved in 50% of cases (Aortic arch and major branch vessels also commonly affected)

Question 26

Takayasu Arteritis (pulseless Disease) was originally described in what population? It is now seen globally.

Answer 26

Takayasu Arteritis was originally associated with Japanese individuals

Question 27

Polyarteritis Nodosa (PAN)
Sx:
Pathogenesis:

Answer 27

Sx: May be highly variable depending upon vessels involved: (Most common) Renal, Heart, Liver, GI (Least Common). Does NOT involve Lungs. “Classic Presentation” : Rapidly accelerating HTN, abdominal pain, bloody stools, myalgias, peripheral neuritis

Pathogenesis: Non-Infectious Vasculitis, likely Immune Complex Mediated. Segmental, transmural, necrotizing inflammation. May see Fibrinoid Necorsis with a predilection for branch points.

Question 28

What Chronic Disease is highly associated with Polarteritis Nodosa (PAN)?

Answer 28

Chronic Hepatitis B is highly associated with Polarteritis Nodosa (PAN). Almost 1/3 of Pt’s with PAN have Chronic Hep B

Question 29

What age demographic would you classically imagine to find Polarteritis Nodosa (PAN) in?

Answer 29

You would classically imagine to find Polarteritis Nodosa (PAN) in younger adults

Question 30

What is the major cause of Mortality in individuals with Polyarteritis Nodosa (PAN)?

Answer 30

The major cause of Mortality in individuals with Polyartertitis Nodosa (PAN) is Rapidly accelerating HTN -> ischemia, infarct, or MI due to Renal Vessel involvement

Question 31

What is the primary method of treatment for individuals with Polyarteritis Nodosa (PAN)? How successful is it?

Answer 31

Immunosuppression is the gold standard treatment for individuals with Polayarteritis Nodosa (PAN), it can yield remission or cures in 90% of patients

Question 32

Kawasaki Disease (Mucocutaneous LN Syndrome)
Sx:
Pathogenesis:

Answer 32

Sx: Erythema of the conjunctiva and oral mucosa with blistering, edema and erythema of the palms and soles, a desquamative rash. Cervical LN enlargement

Pathogenesis: Noninfectious Vasculitis typically involving the Coronary Arteries. May cause Aneurysms, Thrombosis, cardiac sequelae, MI, or sudden death

Question 33

What age demographic is Kawasaki Disease (Mucocutaneous LN Syndrome) typically seen in?

Answer 33

80% of patients with Kawasaki Disease (Mucocutaneous LN Syndrome) are less than 4 years old. Typically infants and small children are affected by this condition

Question 34

What intervention would you use for a Patient with Kawasaki Disease (Mucocutaneous LN Syndrome)?

Answer 34

In a patient with Kawasaki Disease (Mucocutaneous LN Syndrome), which most often would be a child, you would want to treat them with IV-Ig and Aspirin to reduce the odds of a cardiac event. Even though you normally would not want to give a child aspirin.

Question 35

Microscopic Polyangitis (Leukocytoclastic Vasculitis/HSN Vasculitis)
Sx:
Pathogenesis:

Answer 35

Sx: Hemoptysis, Hematuria, Proteinuria, Bowel pain/bleeding, muscle pain/weakness, palpable cutaneous Purpura

Pathogenesis: Non-Infectious Vasculitis. Presents similar to PAN, EXCEPT in Microscopic Polyangiits all lesions tend to be more widely distributed and of the same age, and there is likely going to be lung involvement

Question 36

What are the key differences between Polyarteritis Nodosa (PAN) and Microscopic Polyangiitis (Leukocytoclastic Vasculitis/HSN Vasculitis)?

Answer 36

Microscopic Polyangiitis (Leukocytoclastic Vasculitis/HSN Vasculitis) is associated with MPO-ANCA (p-ANCA), manifests in the lungs, manifests in Venules, and often has Fragmented PMN’s (Neutrophils)

Question 37

Chung-Strauss Syndrome (Allergic Granulomatosis and Angiitis)
Sx:
Pathogenesis:

Answer 37

Sx: Many organ systems may be involved. Cutaneous Palpable Purpura, Gi Bleeding, and renal Disease. Renal involvement may give rise to cardiomyopathy, Heart is involved in 60% of cases and accounts for half of the deaths of Pt’s with this.

Pathogenesis: Non-Infectious Vasculitis, typically of small vessels. Inflammation may resemble PAN or Microscopic Polyangiitis, but will also have Eosinophils and Granuloma formation. Associated with Asthma, Allergic Rhinitis, and Hypereosinophilia. Less than 1/2 show MPO-ANCA positivity

Question 38

Behcet Disease
Sx:
Pathogenesis:

Answer 38

Sx: Classic Triad of symptoms includes Apthous Oral ulcers, Genital ulcers Uveitis. GI and Pulmonary manifestations may be seen as well.

Pathogenesis: Non-Infective Vasculitis. Associated with HLA-B51. Primarily Neutrophillic inflammation. Mortality associated with Aneurysm rupture or Neurological involvement.

Question 39

What treatment is effective for Pt’s with Behect Disease?

Answer 39

Immunosupression with steroids and TNF-Antagonist drugs is effective for Pt’s with Behcet’s Disease

Question 40

What treatment is given for Microscopic Polyangiitis (Leukocytoclastic Vasculitis/HSN Vasculitis)? How effective is it?

Answer 40

Immunosupression is used to treat Pt’s with Microscopic Polyangiitis (Leukocytoclastic Vasculitis/HSN Vasculitis), it induces remission and markedly improves long-term survival

Question 41

Granulomatosis with Polyangiitis (Wegener Granulomatosis)
Sx:
Pathogenesis:

Answer 41

Sx: Focal, Necrotizing, Crescentic Glomerulonephritis. Persistent Pneumonitis and Sinusitis, Renal Disease, and Nasopharyngeal Ulceration. Sinonasal and Pharyngeal Inflammation w/ Geographic Patterns of central necrosis. Cavitation lesions in Lungs

Pathogenesis: Non-Infective Vasculitis. T Cell HSN, Up to 95% of cases associated with PR3-ANCA (c-ANCA). Necrotizing Granulomas in the upper and lower Respiratory tracts

Question 42

How fatal is Granulomatosis with Polyangiitis (Wegener Granulomatosis) if left untreated? How do we treat this disease now?

Answer 42

Granulomatosis with Polyangiitis (Wegener Granulomatosis) is rapidly fatal if left untreated with up to 80% of Pt’s dying in the first year.

We treat it now with steroids, TNF-Antagonists, and Cyclophosphamide

Question 43

Thromboangiitis Obliterans (Buerger Disease)
Sx:
Pathogenesis:

Answer 43

Sx: Cold induced Raynaud’s Phenomenon, Intermittent Claudication, chronic ulcerations -> Gangrene

Pathogenesis: Non-Infectious Vasculitis. Segmental, Thrombosing, Acute and Chronic Vasculitis of small and medium vessels. Frequently involves the Radial and Tibial A.,

Question 44

What does it mean that ANCA’s are “Pauci-Immune”?

Answer 44

Pauci-Immune is used to describe ANCA’s as they are Ab’s against intracellular constituents, and thus will not form Ab-Ag immune complexes in serum

Question 45

What is the difference between Primary and Secondary Raynaud’s?

Answer 45

Primary Raynaud’s happens idiopathically. Typically is in young women, typically symmetrical. Benign course, typically triggered by cold/emotion.

Secondary Raynaud’s is usually the result of another Arterial disease (such as SCLERODERMA, SLE, Buerger’s Disease), typically is asymmetric, worsens with time

Question 46

What is Takotsubo Cardiomyopathy?

Answer 46

Takosubo Cardiomyopathy or “Broken Heart Syndrome” is an occurrence of Myocardial Vessel Vasospasm due to emotional duress that may lead to ischemia or infarct

Question 47

What is the significance of Mucin-producing Adenocarcinomas when it comes to Thrombophlebitis?

Answer 47

Mucin-Producing Adenocarcinomas (typically of the Lung, Ovary, Pancreas) put the body in a state of hypercoagulability due to Mucin being Thrombogenic. This increases the risk of Venous Thrombosis