Vascular Pathology

Front 1

Types of Large Vessel Vasculitis

Back 1

1) Temporal Giant Cell Arteritis

2) Takayasu Arteritis

Front 2

Types of Medium Vessel Vasculitis

Back 2

1) Polyarteritis Nodosa

2) Kawasaki Disease

3) Buerger Disease

Front 3

Types of Small Vessel Vasculitis

Back 3

1) Wegener Granulomatosis (Granulomas w/ Polyangiitis)

2) Microscopic Polyangiitis

3) Churg-Strauss Syndrome

4)Henoch-Schonlein Purpura

Front 4

MOST COMMON vasculitis in older adults

Back 4

Temporal Giant Cell Arteritis

(>50) usually affects Females

Front 5

Temporal Arteritis clinical symptoms?

Back 5

Affects large branches of carotid artery leading to headache (temporal artery), and visual disturbances(ophthalmic artery) and jaw claudication

Some pts. have flu like symptoms w/ joint/muscle pain = polymyalgia rheumatica -> Elevated ESR (>100)

Vascular lesions are SEGMENTAL so a negative biopsy does NOT exclude disease

Front 6

What is at risk to be damaged if Giant Cell Arteritis is not treated?

How is it Tx?

Back 6

Ophthalmic Nerve ischemic damage causing blindness

Tx. w/ corticosteroids

Front 7

Takayasu Arteritis:

type of arteritis?

demographic?

Back 7

Granulomatous vasculitis, classically involves aortic arch at branch points

Young Asian females < 50 years old

Front 8

How does Takayasu Arteritis present? How is it treated?

Back 8

• visual and neurologic symptoms with weak or absent pulse in upper extremities ("pulseless disease")
• ESR elevated

Tx. w/ corticosteroids

Front 9

Polyarteritis Nodosa

Back 9

• Necrotizing vasculitis of MULTIPLE ARTERIES (Poly-arteritis)

• Multiple Arteries -> Involvement of MOST Organs (BUT LUNGS Spared)

• young adults

Front 10

Polyarteritis Nodosa clinical symptoms? Classic finding? a/w?

Back 10

Depends on Arteries Involved

• Hypertension if Renal artery involvement,

• Abdominal pain with melena (Mesenteric artery involvement)

• Neurologic symptoms & Skin Lesions

• HBs (Hep B Surface Ag often found in these pts.)

• 'String of Pearls' appearance on imaging

Front 11

Diseases associated with 'String of pearls'

Back 11

Polyarteritis Nodosa
Fibromuscular Dysplasia

Front 12

What drug treats Polyarteritis Nodosa vasculitis?

Back 12

1) Corticosteroids (stop Inflammation)

2) Cyclophosphamide

FATAL if NOT Treated

Front 13

Kawasaki Disease demographic

Back 13

Asian children < 4 years old

Front 14

Kawasaki Disease presentation

Back 14

Non-specific symptoms:

Fever

Conjunctivitis

Erythymatous Rash on palms and soles

Lymphadenopathy of Cervical Lymph nodes

Front 15

Which artery is commonly involved in Kawasaki?

Back 15

Coronary Artery; risk of

(1) thrombosis with MI

(2) aneursym with rupture

Front 16

Kawasaki Disease treatment

Back 16

1) Aspirin

2) IVIG (IVIG reduces vasculitis in KD by suppressing the marked immune activation associated with this disease b/c it upregulates Suppressor Tcells that block release of IFN-Gamma/IL-1 & TNF that drive the pathophysiology of Kawasaki's)

Front 17

Buerger Disease HY associations

Back 17

SMOKING disease

Tx = STOP Smoking

presents with Necrotizing vasculitis involving Digits (Fingers & Toes). Associated with Raynauds (vaso-spasm of blood vessels going to digits) (Turn White (b/c blood supply cut off)->Blue (b/c tissue becomes cyanotic)->Red (when blood flow returns after smasm ends)) ->autoamputation of digits w/out Tx.

Front 18

Wegener's Granulomatosis

Back 18

• Necrotizing granulomatous vasculitis involving nasopharynx, lungs and kidneys.
• c-ANCA positive
• Treatment is Cyclophosphamide
• large necrotizing granulomas with adjacent necrotizing vasculitis on biopsy

Front 19

Classic presentation in Wegener's granulomatosis

Back 19

Middle aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis

Front 20

Wegener's Granulomatosis

vs.

Microscopic Polyangiitis

Back 20

Same

1) Both involve multiple (Poly-angitis)

2) Both involve Lung & Kidney

3) Tx. Cyclophosphamide & Corticosteroids

4) Relapses are common

Different: Unlilke Wegener's Granulomatosis, Microscopic Polyangitis has:

1) No Nasopharyngeal involvment or granulomas

2) serum p-ANCA (p-ANCA levels correlate w/ severity of Dz.) NOT c-ANCA

Front 21

Microscopic Polyangiitis vs. Churg-Strauss Syndrome

Back 21

Same

1) Involves multiple organ systems

2) Involve LUNGS -> particularly LUNGS (Churg-Strauss)

3) p-ANCA +

Different: Unlike Microscopic Polyangitis, Churg-Strauss has:

1)Necrotizing Granulomatous Vasculitis w/

2) Eosinophils (peripheral eosinophilia) leading to...

3) Asthma

4) Involves Heart

Churg-Strauss presents with Asthma, Granulomas and peripheral eosinophilia.

They BOTH have lung involvement, both express p-ANCA.

Front 22

Henoch-Schonlein Purpura

Back 22

Vasculitis due to IgA Immune complex deposition

Presents with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy); usually occurs following UTI

Front 23

Henoch-Schonlein Purpura demographic

Back 23

most common vasculitis in children

Front 24

Important cause of stenosis in young females?

Back 24

fibromuscular dysplasia (frequently involves renal artery) - developmental defect of blood vessel wall, resulting in irregular thickening of large and medium sized arteries

Front 25

Primary HTN

Back 25

HTN of unknown etiology (95% of cases)

Front 26

Secondary HTN

Back 26

due to identifiable etiology (ie renal artery stenosis, atherosclerosis, fibromuscular dysplasia)

Front 27

Malignant HTN

Back 27

severe elevation in BP ( >180/120 mm Hg):

1. may arise from preexisting benign HTN or de novo

2. presents with acute end-orange damage (eg acute renal failure, headache, and papilledema) and is medical emergency

Front 28

Atherosclerosis

Back 28

thickening of INTIMA, affects medium/large sized arteries (abdominal aorta, coronary artery, popliteal artery, internal carotid artery)

Front 29

Complications of atherosclerosis

Back 29

1. Stenosis of medium-sized vessels leadings to peripheral vascular disease, angina, ischemic bowel disease

2. Plaque rupture with thrombosis resulting in MI and stroke

3. Plaque rupture with embolization

4. Aneurysm

Front 30

Hyaline arteriosclerosis

Back 30

caused by proteins leaking into vessel wall, producing vascular thickening; proteins are seen as pink hyaline on microoscopy

Front 31

Causes of hyaline arteriosclerosis

Back 31

long standing benign HTN or diabetes

Front 32

Hyperplastic arteriolosclerosis

Back 32

thickening of vessel wall by hyperplasia of smooth muscle ('onion-skin' appearance) due to malignant HTN

Front 33

What does hyperplastic arteriosclerosis lead to?

Back 33

reduced vessel caliber with end-organ ischemia; may lead to fibrinoid necrosis of vessel wall with hemorrhage; classically causes acute renal failure with 'flea-bitten' apperance

Front 34

Complications of aortic dissection

Back 34

Obstruction of branching arteries results in end-organ ischemia

Front 35

Most common cause of death due to aortic dissection

Back 35

pericardial tamponade

Front 36

Classically causes thoracic aneurysm

Back 36

tertiary syphilis which causes endartertis of the vaso vasorum resulting in 'tree barkin' of the aorta.

Front 37

Major complication of thoracic aneurysm

Back 37

dilation of the aortic valve root, resulting in regurgitation; other complications include compression of mediastinal strucutres (eg airway or esophagus) and thrombosis/embolism

Front 38

Monckeberg medial calcific sclerosis

Back 38

calcification of media of muscular arteries; nonobstructive; not clinically significant (incidental finding on X ray)

Front 39

Aortic dissection

Back 39

Intimal tear with dissection of blood through media of aortic wall; occurs in proximal 10 cm of aorta with preexisting weakness of media

Front 40

Causes of aortic dissection

Back 40

1. HTN (most common)

2. Inherited defects of connective tissue (Margan or Ehlers-Danlos syndrome)

Front 41

Kaposi's Sarcoma demographic

Back 41

Older Eastern European males -- tumor remains localized to skin

AIDS and Transplant patients -- tumor spreads early.

Front 42

Abdominal aortic aneurysm

Back 42

Balloon-like dilation of abdominal aorta; usually arises below renal arteries but above aortic bifurcation

Front 43

Causes of abdominal aortic aneurysm

Back 43

Atheroslcerosis; classically seen in male smokers >60 with hypertension

Front 44

Hemangioma

Back 44

Benign tumor comprised of blood vessels; commonly present at birth and regresses during childhood; often involves skin and liver

Front 45

Angiosarcoma

Back 45

• Malignant proliferation of endothelial cells; highly aggressive
• Common sites: skin, breast, liver

Front 46

What is liver angiosarcoma associated with?

Back 46

exposure to polyvinyl chloride, arsenic, and Thorotrast

Front 47

Kaposi sarcoma

Back 47

Malignant proliferation of endothelial cells; associated with HHV-8

Front 48

How does Kaposi sarcoma present?

Back 48

Purple patches, plaques, nodules on skin; may also involve visceral organs

Front 49

Modifiable Risk factors for Atherosclerosis

Back 49

1) HTN

2) Hypercholesteremia

3) Smoking

4) Diabetes

All these drive Pathophysiology fo Atherosclerosis

Front 50

Non-Modifieable Risk factors for Atherosclerosis

Back 50

1) Age - # of lesions Incr. w/ age

2) Gender - Males >> than pre-menopausal Females (b/c estrogen protective)

3) Genetics - Family History highly predictive od risk

Front 51

Pathogenesis of Atherosclerosis

Back 51

Intima is Endothelial cells sitting on BM

1) Lipid Invasion of Intima = Endothelial damage allows lipids to interact w/ BM

2) The lipids are oxidized

3) Oxidized lipids Phagocytosed by Macrophages (Macrophages called in to clear these oxidized lipids)

4) Collection of Macrophages filled with lipids are now in Intima = FATTY STREAK - yellow streaks running along large blood vessels - already seen in teenagers

5) Macrophages activate Inflammation, followed by healing-> Fibromuscular cap

6) Each bout of inflamamtion, brings more lipids into the intima->more lipid laden Macrophages->More inflmmation. Therefore the atherosclerotic plaque continues to grow if there is Increased inflmmation and LDL cholesterol in blood

7) Fibromuscular cap is part of the Healing Process, as deposition of ECM (granulation tissue), proliferation of smooth muscle (both can be done by fribroblasts)

8) As the plaque expands, it increasingly occludes blood flow