Exam IV: Hypertension & Vessel Diseases I

Question 1

Essential Hypertension

Answer 1

Also known as Primary Hypertension
95% of cases and idiopathic/unknown cause
Multifactorial: enetic polymorphisms and environmental factors
Usually does not cause short-term problems
If controlled: compatible with long life and asymptomatic

Mechanisms:
Genetic factors
Reduced renal sodium excretion
Vasoconstrictive influences
Environmental factors

Question 2

Primary vs. Secondary Hypertension

Answer 2

Essential = Primary Hypertension: means that it is the initial presentation of any disease
Secondary: developed from a disease that the patient had first

Question 3

Primary/Essential Hypertension

Answer 3

Primary 90-95% of patients
Environmental, Lifestyle, Genetic
No single gene identified, but substantial genetic component - 32-57%
Western diet lifestyle increases risk

Question 4

Secondary Hypertension

Answer 4

Secondary = 2-5% of patients
Renovascular
Other Renal
Endocrine: endogenous or exogenous (OCP/oral contraceptive pill, 5% develop hypertension)

Renal Artery Stenosis- #1 cause
90% - Atherosclerotic in aorta blocks the renal arteries
Usually > 50 of age and smoke
10% Fibromuscular dysplasia- usually young women

Question 5

Secondary Hypertension: Fibromuscular Dysplasia

Answer 5

Fibromuscular Dysplasia -tends to affect young women (20s and 30s)

Pathology
Intimal and medial layers affected (most common) with adventitial subtypes
Distal arterial segmental stenosis

String of beads sign – intimal thickening in certain areas and not in other areas causing the beads formation
Just change in vasculature

Question 6

Secondary Hypertension: Endocrine

Answer 6

Hyper or hypothyroidism

Adrenal Adenoma/Hyperplasia:

1. Aldosteronoma
2. Cushings syndrome
3. Pheochromocytoma

Primary disease in endocrine organs leading to secondary hypertension

Question 7

Secondary Hypertension: Vascular

Answer 7

Coarctation of the aorta
Narrowing just distal to subclavian- causes damage to surface
Typically upper limb hypertension with lower extremity gradient

Occurs in Turner’s syndrome

Key clinical point- hypertension in upper extremities, but lower extremities are normal

Question 8

Myocardial and Renal Effects of Hypertension

Answer 8

Myocardial– cardiac hypertrophy, ischemic injury because pushing against high pressure, compensation for overloading/high volume

Vascular and Renal:

1. Hyaline arteriolosclerosis and arterial sclerosis (benign nephrosclerosis)
2. Proliferative arteriolosclerosis (onion-skinning) and fibrinoid necrosis (malignant nephrosclerosis/hypertension)

Question 9

Hypertensive Emergency(Malignant Hypertension)

Answer 9

Severe hypertension causing acute organ impairment
CNS, KIDNEY, HEART
Pathologically with more severe vessel damage, often thrombus (thrombotic microangiopathy) and fibrinoid necrosis of vessel wall
Since turbulence in vessels = clot formation
Fibrinoid necrosis of vessel wall = start to see a lot of problems like ischemia, thrombosis, etc.

Question 10

Accelerated (Malignant) Hypertension

Answer 10

~5% of hypertensives
Rapidly rising blood pressure
Untreated: death within a year or two because continuous under perfusion of organs

Severe hypertension:
Systolic >200 mm Hg
Diastolic >120 mm Hg)

Renal failure
Retinal hemorrhages and exudates with or without papilledema
Superimposed on pre-existing benign hypertension

Question 11

Malignant Hypertension: Kidneys and Thrombotic Microangiopathy

Answer 11

Flea – bitten kidney in malignant hypertension; petechial hemorrhages due to areas of fibrinoid necrosis of vessels

Thrombotic microangiopathy may be seen microscopically:
No more nice vessel wall- broken down
Clot formation in lumen

Question 12

Hypertension in the Retina

Answer 12

Retina: narrowing of vessels causing hemorrhages within the eyes

Question 13

Hypertension in the Brain

Answer 13

Intracerebral Hemorrhage
Mid to late life – peak in 60s
Rupture of small intraparenchymal vessel
Hypertension accounts for >50%

Charcot- Bouchard Microaneurysms- small aneurysms of small vessels usually in the basal ganglia

Just one vessel to cause a big hemorrhage
Bleeding in the brain- drill holes to release pressure and bleeding

Lacunar Infarcts: lake-like spaces (not more than 15 mm)

Question 14

Hypertensive Encephalopathy

Answer 14

Hypertensive Encephalopathy: presentation of hypertensive emergency

Clinical pathologic entity: severe cerebral dysfunction and pathologic correlates of cerebral edema and fibrinoid necrosis of arterioles

Fibrinoid necrosis: deposition of Ab-Ag complexes in the vessel walls (antibody antigen complexes)

Question 15

Regulation of Normal Blood Pressure

Answer 15

Blood pressure: function of cardiac output and peripheral vascular resistance

Influenced by multiple genetic, environmental, and demographic factors

Major factors that determine blood pressure variation:
BMI and diet = modifiable
Age and gender = cannot modify

Question 16

Hypertension: Small Blood Vessel Disease

Answer 16

Hypertension: associated with 2 forms of small blood vessel disease

1. Hyaline arteriolosclerosis
2. Hyperplastic arteriolosclerosis

Question 17

Hyaline Arteriolosclerosis

Answer 17

Morphology:
Homogeneous, pink hyaline thickening
Luminal narrowing
Generalized and severe in individuals with hypertension
Common feature of diabetic microangiopathy

Deposition of hyaline (pink stuff) with nice nuclei, but in fibrinoid necrosis you won’t see nice nuclei

Question 18

Hyperplastic Arteriolosclerosis

Answer 18

Morphology:
“Onion-skin” lesions- concentric laminated thickening of walls and luminal narrowing
Smooth muscle cells and reduplicated basement membranes

Occurs in severe (malignant) hypertension (kidney)
“Onion-skin” lesion plus fibrinoid deposits
Vessel wall necrosis (necrotizing arteriolitis)

Question 19

Aneurysm

Answer 19

Aneurysm: atherosclerotic plaque in intima layer pushes on media layer and starts to die because prevents blood and nutrients from getting through as well as the waste material from exiting
Causes necrosis of media/breakdown of media and wall gets weak and thin so bulges out

Most common in abdominal aorta

Question 20

Classifications of Aneurysms

Answer 20

True – contains all layers of the vessel wall
False (pseudo) – contained rupture

Fusiform – circumferential and symmetric
Saccular – asymmetric bulging of a portion of the wall (one sided)

Dissection

Question 21

Aortic Dissection

Answer 21

Intramural blood between inner 2/3 and outer 1/3 of aorta
Between the intima and media: because small break in intima and zipper stars to spread out
May or may not be associated with aortic dilation

Question 22

Cystic Medial Degeneration

Answer 22

Pooling of mucosubstances, elastic fragmentation with disruption of elastic lamellar structure, fibrosis and loss of smooth muscle cell nuclei
Media degenerates and breaks down
Seen in Marfan’s syndrome

Question 23

Aortic Aneurysm

Answer 23

Localized permanent pathologic dilatation of a vessel at least 50% >normal

What is normal?
Relates to age, location, body habitus and daily workload
Increase age = increase diameter
Ascending normal <3.0 cm

Question 24

Complications of Aortic Dissection

Answer 24

1. Rupture or tear of vessel and can get into the pericardium
2. Within pericardium, only supposed to be 15mL fluid in there, but the blood leaking in causes blood all around the heart in the pericardium and will clot and cause cardiac temponade so heart cannot beat and stops beating
3. Causing collapse of coronary arteries

Either way you cut it… not a good thing to have

Question 25

Marfan's Syndrome

Answer 25

Tend to see aortic dissections in Marfan’s syndrome because mucosubstance build up in the media layer Fibrillin gene mutation causes breaks/disruptions in elastic bands (stars in photo A) Mucopolysubstances – get intima damage/tears, separate and then aortic dissection

Question 26

Peripheral Arterial Diseases

Answer 26

Large vessels: aorta; diseases include granulomatous disease with giant cell arteritis and Takayasu arteritis Medium: branches off of aorta and arteries; diseases include immune complex mediated such as polyarteritis nodosa, and anti-endothelial cell Ab such as Kawasaki disease Small: arterioles, capillaries, venules, and veins

Question 27

Giant Cell (Temporal) Arteritis

Answer 27

Most common form of vasculitis among elderly individuals in the United States and Europe Chronic, granulomatous inflammation of large to small-sized arteries: Arteries in the head, temporal arteries, and vertebral and ophthalmic arteries Nodular intimal thickening: see altering/skip lesions (makes bead formation) to reduce diameter and blood flow isn’t going where is needs to causing transient ischemic changes Classic lesions: see granulomatous inflammation (giant cells), but if you don’t see this stain the elastic lamina and if fragmentation present then know it is temporal arteritis

Question 28

Takayasu Arteritis

Answer 28

Also known as granulomatous vasculitis of medium and larger arteries Ocular disturbances Marked weakening of the pulses in the upper extremities AKA pulseless disease Transmural fibrous thickening of the aorta Severe luminal narrowing of the major branch vessels Adventitial mononuclear infiltrates are perivascular, so cuffing of the vasa vasorum Intense mononuclear inflammation in the media Granulomatous inflammation: giant cells and patchy medial necrosis

Question 29

Polyarteritis Nodosa

Answer 29

Systemic vasculitis of small or medium-sized muscular arteries Not arterioles, capillaries, or venules Involving renal and visceral vessels but sparing the pulmonary circulation, thus it DOES NOT AFFECT THE LUNGS Microscopically: Areas of un-involved tissue aka has segmental involvement Fibrinoid necrosis

Question 30

Kawasaki Disease

Answer 30

Leading cause of acquired heart disease in children Acute febrile, self-limited illness Associated with an arteritis: affects large to medium-sized and small vessels Predilection for coronary artery involvement Pronounced inflammation affecting the entire thickness of the vessel wall The child is ill or just recovered from an illness… have fever if ill

Question 31

Microscopic Polyangiitis

Answer 31

Necrotizing vasculitis Affects capillaries, arterioles and veins AKA hypersensitivity vasculitis or leukocytoclastic vasculitis Necrotizing glomerulonephritis and pulmonary capillaritis--particularly common Segmental fibrinoid necrosis of the media with focal transmural necrotizing lesions INVOLVEMENT OF THE LUNGS

Question 32

Churg-Strauss Syndrome

Answer 32

AKA allergic granulomatosis and angiitis Relatively rare Small-vessel necrotizing vasculitis Associations: asthma, allergic rhinitis, lung infiltrates, peripheral hypereosinophilia, extravascular necrotizing granulomas Vascular lesions: Similar to polyarteritis nodosa or microscopic polyangiitis Accompanied by granulomas and eosinophils Other clinical findings: Palpable purpura Gastrointestinal tract bleeding Renal disease (focal and segmental glomerulosclerosis)

Question 33

Wegener Granulomatosis

Answer 33

New name: Granulomatosis with polyangiitis Necrotizing vasculitis: vessel walls fall apart and blood surround tissue and alveolar hemorrhage Triad: 1. Acute necrotizing granulomas of the URT or LRT or both 2. Necrotizing or granulomatous vasculitis affects small to medium-sized vessels 3. Renal disease: focal necrotizing, often crescentic, glomerulonephritis

Question 34

Wegener Granulomatosis: Lesions

Answer 34

Lung lesions: Granulomas with geographic patterns of central necrosis = caseous Vasculitis Alveolar hemorrhage Renal lesions: Focal and segmental necrotizing glomerulonephritis Crescentic glomerulonephritis Middle aged person without eosinophils = Wegener’s case Intermittent coughing up blood

Question 35

Thromboangiitis Obliterans

Answer 35

AKA Buerger’s disease Almost exclusive to heavy cigarette smokers Before the age of 35 Medium-sized and small arteries: tibial and radial arteries Segmental and thrombosing Acute and chronic inflammation Luminal thrombosis: small microabscesses Claudication symptoms or cramping in lower legs because reduction in lumen in lower extremities and the blood flow isn’t adequate Sometimes patients must have amputation of fingers or legs

Question 36

Raynaud Phenomenon

Answer 36

Exaggerated vasoconstriction of digital arteries and arterioles Characteristic changes: digits show red, white, and blue color changes; most proximal to most distal

Question 37

Secondary Raynaud Phenomenon

Answer 37

Secondary Raynaud phenomenon Vascular insufficiency of the extremities secondary to arterial disease caused by other entities including: SLE, scleroderma, Buerger disease, or atherosclerosis