Blood Vessels

Question 1

Arteries & Arteriosclerosis

Answer 1

• From inner to outer layers -
  
  • Intima (endo cells)
  • Basement membrane (internal elastic lamina)
  • Media (smooth muscle)
  • Adventitia (anchor vessel)
• Sclerosis = Hardening of artery/arterioles
• Thickening & LOSS of elasticity of BV wall
• AterioloSclerosis-Hyaline assoc w/Kidney
• 3 forms:

1. **Monckeberg medial calcific **
2. Athero
3. Arteriolo ^{(associated w/Hypertension & Diabetes)}

Question 2

Arteriosclerosis-Monckeberg Medial Calcific

Answer 2

• Calcium deposits found in the tunica media of muscular arteries
• In pelvis, upper limbs, uterus in ages ABOVE 50
• Dytrophic calcification ^{(no clinical significance)}
• Non-occulsive = No affect on blood flow
• Diagnosed w/Xray or mammogram
• Symptoms:
  
  • Arterial stiffness
  • High pulse pressure
  • Damage to Heart/Kidney

Question 3

Atherosclerosis (ATH)

Answer 3

• Definition: migration of smooth muscle cells from media to intima of large to medium sized arteries
• Media of arterty is thinned out
• Intimal injury = Intimal thickening, fatty streak ^{(macrophages)}, Ingested LDL ^{(Foam cells)},Migration = intimal thickening LEADS to degradation of collagen/elastin
• Intimal atheromatous or *fibro-fatty plaque:*
  
  • Fibrous cap (smooth muscle cells, foam cells, neovascularization-Sprouting of new BVs from exsisting ones)
  • Necrotic center-cholesterol crystals & calcium
• Atheromatous turns into complex lesion AKA Rupture/Thrombus

Question 4

Risk Factors & ATH

Answer 4

Risk factors:

• Potentially controllable-
  
  • LDL cholesterol
  • Diabetes
  • Hypertension
  • Smoking
  • Hyperlipidemia ^{(HIGH LDL, LOW HDL or defective LDL receptor)}
  • Chlamydia
• Non-controllable-
  
  • Increasing age
  • Males & post menopausal (estrogen def)
  • Family history
  • Physical inactivity

Question 5

Stages of ATH

Answer 5

• Normal to Fatty streak-
  
  • Endothelial dysfunction
  • Monocyte adehesion
  • SMC migration to intima
  • SMC proliferation
  • ECM elaboration
  • Lipid accumulation
• Fibrofatty plaque to Adv vulnerable plaque-
  
  • Cell death/degeneration
  • Inflammation
  • Growth & remodeling of plaque/ECM
  • Organization of thrombus w/calcification
• Mural thrombis embolization wall weakened = aneurysm/rupture
• Plaque rupture, erosion, hemmorhage = Occulsion by thrombus
• Plaque growth = Critical stenosis

Question 6

Morphology of Atheroma

Answer 6

• Location:
• Most common abdominal aorta
• Thoracic aorta
• Circle of Willis
• Renal & mesenteric arteries
• Rupture, ulceration or erosion = Thrombus formation
• Hemorrhage (coronary arteries) = Thrombus formation
• Calcification = Dystrophic type ID with X-ray
• Aneurysmal dilation = Atrophy of smooth muscle of media/LOSS of elastic tissue
  
  • Pulsating mass in abdomen (below renal artery)
  • Rupture & hemm shock = Sudden loss of BP, cool skin, Loss of consciousness

Question 7

Clinical Signs of ATH

Answer 7

• Symptoms due to LOW tissue O2:
  
  • Coronary artery-Pain w/exercise
  • Brain-TIA & stroke
  • GIT-Mesenteric artery ATH=Ischemia of intestine ^{(postprandial pain-Pain after eating & weight loss)}
• Lower extremities-ATH of popliteal:
  
  • Gangrene of legs
  • Impotence
  • Pain on walking
• Renal artery (bilateral):
• Elevated renin/aldosterone = Secondary hypertension

Question 8

Hypertension

Answer 8

• Sustained diastolic BP above 90mmHg or systolic pressure in excess of 140mmHg
• Malignant hypertension = Diastolic greater than 120 / Systolic greater than 200
  
  • ex. Pheochromocytoma
• Risk factor for:
• CAD (coronary artery disease)
• CVA or Stroke (cerebro-vascular accident)
• Idopathic HT:
  
  • 90-95% compatible w/long life
  • Family history IMPORTANT
  • Present for 10-20years
• Secondary HT:
  
  • 5-10%
  • Related to renal issues (renal hypo-perfusion)
  • Sudden presentation

Question 9

Primary (essential) Hypertension

Answer 9

• Aka-Benign HTN = Long standing
• Family history present
• No short term problems
• Compications from MI or CVD
• Salt-sensitive hypertension w/normal renin activity
• Genetic factors-Single gene disorder
  
  • Increase aldosterone secretion
  • Defect in renal Na+ homeostasis
• Increases to-Cardiac output, peripheral resistance & ECF = Hypertension

Question 10

Secondary HTN

Answer 10

• Renal:
  
  • Chronic renal disease
  • Renal artery stenosis
  • Renin producing tumors (renal cell carcinoma)
• Endocrine:
  
  • Pheochromocytoma = Increase in catecholamines
  • Conn’s syndrome = Adenoma in adrenal cortex (increase in aldosterone w/decrease in renin)
  • Cushing syndrome (high lvls or cortisol)
• Cardiovascular: Coarction of aorta=Upper limb HTN & Poly arteritis Nodosa ^{(vasculitis w/clinical sign “rosary beads”)}
• Neurological: increased intracranial pressure

Question 11

Renal artery stenosis (Fibro-muscular)

Answer 11

• Can be unilateral greater chance in females
• Histo: Deposition of trichrome positive collagen in artery wall w/Medial hyperplasia ^{(Beads on a string)}
  
  • Found in lamina elastic interna
• Hypoxia = High renin & aldosterone in serum of affected vessels
  
  • Net effect = Small atrophic kidney
• Malignant Hypertension =Visual Kidney changes

Question 12

Malignant Hypertension

Answer 12

• Hyaline arteriolosclerosis:
• Arteriolar wall becomes hylinized = Narrow lumen
• Kidney = Fine granular change (benign nephrosclerosis)=Severe forms seen Diabetes
• Histo: Pink glossy in wall
• Hyperplastic = onion skinning leads to luminal obliteration (ischemia-wrinkling of glom capillary vessels)
• Kidney = multiple hemorrhages (flea-bitten) assoc w/glomerular damage
  
  • Can be assoc w/Fibrinoid necrosis - Deposits of eosinophilic material in intima

Question 13

Clinical signs of Malignant HTN

Answer 13

• Palpitation
  
  • Irregularly irregular HB
  • Brought on by peripheral edema=CHF
• Blurring vision
• Headache
• Emergency HTN:
• Renal failure (hematuria)
• Retinal hemorrhages
• Papilledema (increased intracranial presure)
  
  • Swelling of optic disk
• Diseases assoc w/HTN:
• Left vent hypertrophy
• Benign nephrosclerosis
• Berry’s aneurysm

Question 14

Aneurysms

Answer 14

• Localized abnormal dialtion of a BV (vein or artery) or dialtion of heart
• Most common causes for aortic:
  
  • Atherosclerosis
  • Hypertension
• Cystic medial degeneration (necrosis):
  
  • Loss of elastic muscle fibers in media & replaced w/basophilic ground substance
  • Marfan syndrome (defective fibrillin needed to make elastic fibers)
• Cong. defects = Berry aneurysms (subarachnoid space hem)
• Syphilis ^{(spirochete bacter)} affects thoracic aorta
• Mycotic = due to infection of major artery

Question 15

Abdominal Aortic Aneurysms

Answer 15

• Most common
• Cause:
  
  • Atherosclerosis
• Location:
  
  • Below renal arteries & above bifurcation of aorta
• Complications:
  
  • Rupture into peritoneal cavity or retro = SHOCK
  • Develop mural thrombus = Lower body embolism (impotence & gangrene)
• BP is crucial in severity of aneurysm

Question 16

Syphilitic (Leutic) Aneurysms

Answer 16

• Location = Thoracic aorta
• Tertiary syphilis:
  
  • Inflammation of adventia
  • Leads to inflammation of Vasa vasorum ^{<strong>(Blood supply of aorta)</strong>}=Obliterative endarteritis
  • Loss of elastic fibers = Ischemia
  • End result dilatation of thoracic aorta
• Appearance: “Tree bark” - wrinkled
• Cardiac disease: Aortic valve dialation or regurg
• Massive left vent hypertrophy & dialation = Cor bovinum
• Diagnose w/Ab for Treponema Pallidum

Question 17

Aortic Dissection General

Answer 17

• Tear in the intima (inner layer) = Intramural hematoma
• Presence of blood between & along the laminar planes of media (outer/middle)
• Causes DILATATION & Dissecting aneurysms
• Histo: Medial degeneration marked by elastin fragmentation (Marfan)=Black lines are scattered not consistant
• Proximal dissection: ascending aorta complete or region of aortic valve ^{<strong>(arch)</strong>}
• Predisposing factors:
• Hypertension, 40-60, any part of aorta
• Marfan:
• Myxoid change & fragmentation of elastic lamina ^{(cystic medial necrosis)}
• Above or AT aortic root

Question 18

Clinical Presentation of Aortic Dissection

Answer 18

• Propagation of SUBintimal hematoma:
  
  • Makes true/false lumen w/in aorta = Double barreled aorta
  • False lumen grows = Decrease amount of blood flow in true
• Dissection w/in pericardial space = Cardiac tamponade
• Dissection @ root = tamponade, wide mediastinum ^{(heart shadow)}, diminished heart sound, SUDDEN collaspe/shock
• ECG: ST segment elevations (vent depolar) can be mistaken w/MI
  
  • ST depression also happens more severe
• Tearing chest pain-Radiate to back
• Shifting pain w/time

Question 19

Giant cell (temporal) Arteritis General

Answer 19

• Acute or chornic granulomatous inflammation w/lymphocytic infiltrate
• Histo:
• Degenerated internal elastic membrane in acute arteritis
• Intimal thickening in chronic or healed arteritis
• Nodular thickening of affected artery
• Involves:
  
  • Temporal artery-Headache
  • Opthalmic-blindness, diplopia
  • External carotid artery-Trans Ischemic attack less than 24 hours

Question 20

Clinical features of Giant cell (temporal)

Answer 20

• After age 50
• Polymyalgia rheumatica (joint/muscle pain) neck & shoulder stiffness/pain
• Jaw pain
• Abrupt headaches
• Weight loss/Anorexia
• Loss of vision
• Lab value: High ESR (sedimenation rate) sticky due to fibronogen inflammation=over 100mmHr
• Corticosteroid therapy sucessful

Question 21

Takayasu Arteritis (Pulseless disease)

Answer 21

• Granulomatous vasculitis autoimmune marker ANCA ^{(antineutrophil cytoplasmic Ab)}
• Destruction of media by mononuclear inflammation
• AKA aortic arch syndrome
• Females younger than 40
• Vessels involved:
  
  • Aortic Arch
  • Renal=^{secondary hypertension}
  • Pulmonary
• Imaging: Narrowing of brachiocephalic, carotid & subclavian arteries
• Morphology: Stenosis & or aneurysm
• Clinical:
• Low BP w/weak pulse in upper (carotid, radial, ulnar) than in lower
• Ocular disturbances-Unilateral blindness

Question 22

ANCA vasculitis

Answer 22

• Anti-neutrophil cytoplasmic Ab
• Wegner granilomatosis = C-anca
• Churg-Strauss = P-anca
  
  • Asthma & eosinophila
  • Fibrinoid necrosis
• Microscopic polyangitis = P-anca
  
  • NO asthma
  • Neutorphil infiltration in BV

Question 23

Polyarteritis Nodosa (PAN)

Answer 23

• Systemic vasculitis (medium vessel)
• Hep B Surface Ag (HBsAg)+++
• ANCA (-)
• Perfusion impairment=^{infarcts-necrosis}
• Involves:
  
  • Kidney
  • Skin
  • Other organs
  • NO LUNG
• Morphology:
  
  • Fibrinoid necrosis (bright pink fibrin Ca+2 deposit)
  • Thrombosis
• Clinical PAN:
• Effects all age groups
• Malaise, fever, weight loss
• HT w/ab pain w/Melena (tar feces)
• *palpable skin purpura *

Question 24

Churg-Strauss Syndrome

Answer 24

• Autoimmune of medium & small vessels w/History of airway allergy sensitivity
• Variant of PAN
• Allergic granuloma invloving PULMONARY vasculature
• Eosinophila = Damage to vessels & necrosis
• Symptoms:
  
  • Asthma
  • Allergic Rhinitis
• Positive for P-ANCA target for myloperoxidase

Question 25

Kawasaki Disease

Answer 25

• Mucocutaneous lymph node syndrome
• Effects young children
• Associated w/Auto-immune or pathogen
• Clinical:(Triad of symptoms)
• Conjunctivitis
• Maculopapular skin rash
• Lymphadenopathy
• Redness on soles/palms
• Location:
• Right & Left Coronary artery leads to MI due to aneurysm

Question 26

Leukocytoclastic Vasculitis

Answer 26

• Microscopic polyangitis-Affects Arerioles, capillaries, venules
• Etiology-reaction to an Ag like:
  
  • Drugs (penicillin)
  • Microorganisms (strep)
  • Malignancy
• Characteristic:
  
  • Deposition of antigen-Ab complex in vessel wall
  • P-ANCA (+) detected by Myeloperoxidase
  • Palpable skin purpura ^{(more common than PAN)}
  • Blood stained sputum ^(hemoptysis)
  • Arthalgia ^{<strong>(joint pain)</strong>}
• Morphology: **Neutrophilic vasculitis **
  
  • Non-granulomatous-neutrophil/lymphocytes

Question 27

Henoch-Schonlein Purpura

Answer 27

• Type of leukocytoclastic vasculitis ID by IgA mediated skin purpura
• IgA deposition in kidney = Hematuria
  
  • Depostition in Glom
• Peri-vascular IgA deposit in skin = DERMATITIS HERPETIFORMIS
• P-ANCA (-)
• Abdominal manifestations:
  
  • Pain
  • Vomiting
  • Intestinal bleeding
  • nonmigratory arthralgia

Question 28

Systemic Lupus Erythematosus

Answer 28

• Fibrinoid necrosis present
• Ab in SLE:
  
  • Anticardiolipin Ab=Deep venous thrombosis (antiphospholipid syndrome)
  • ds-DNA & Anti-smith
• Pts w/o C2-C4 are more susceptable to SLE
• Hep-B pts more susceptible to Fibrosis

Question 29

Wegner Granulomatosis

Answer 29

• Necrotizing vasculitis - affecting capillaries, venules, arterioles, & arteries
• Morphology-Giant cells in lung/kidney Blood vessels
• Nodular infiltrative lesions in lung
• Affects BOTH upper/lower
• Causes palatal destruction
• Death w/in 1 year if untreated
• c-ANCA (+) in seurm 95% and can monitor disease progression
• Nasopharynx-Mucosal ulceration, sinusitis, rupture of nasal septum=^{<u>Bleeding</u>}
• Lung- pneumonitis
• Kidney:
• Crescentic glomerulonephritis
• acute renal failure
• Secondary Hypertension

Question 30

Raynaud Phenomenon

Answer 30

• Primary (disease) = Vasoconstriction due to cold, stress ^{<strong>(neurological)</strong>}
• Secondary (phenomen) = Pathological obstruction of artery
  
  • Buerger
  • Scleroderma (fibrosis)
  • SLE
• Burger = Claudication (trouble walking) of feet
• Affects tibial & raidal artery
• Color change = White-Blue-Red
• Kidney **meningioma present **

Question 31

DVT

Answer 31

• Cause = Hypercoagulable states
  
  • Adenocarcinoma (colon, lung, urogenital)-Can start off as trousseau syndrome
  • Pregers
  • Immobilization
  • Bone fracture
• Vessels: Iliac, femoral, Popliteal MOST common
• Saddle thrombus ->Acute cor pulmonale (RHF)->pulmonary infarction = Acute dyspnea (Quick death)

Question 32

Benign Vascular Tumors (Spider Telangictasia)

Answer 32

• Arterial Spider
• Radial & often pulsatile
• Array of dilated subcutaneous arteries or arterioles w/central core
• Etiology:
  
  • Hyperestrinism seen in liver failure due to liver not breaking down estrogen
  • Gyenecomastia SE ^(males)
  • Cirrhosis w/portal HTN

Question 33

Hereditary Hemorrhagic telangiectasis

Answer 33

• Osler-weber-rendu disease (angiodysplasia)
• Autosomal dominant
• _Morphology: _
• Vascular deformation = meshwork dilated capillaries, veins, arteries
• Arterio-venous malformation = Hamartoma ^{<strong>(thick wall artery & thin wall veins together)</strong>}
• Location:
• Present @ birth
• Distrubuted widely over skin & mucous membranes
• Clinical presentation:
• Spontaneous nosebleeds
• Bleeding into gut/rectum
• Hematuria

Question 34

Hemangiomas

Answer 34

• Capillary hema:
  
  • Benign BV
  • Found on skin rarely visceral organ (interior organ)
  • Red skin nodule (strawberry)
  • Regresses spontaneously
• Cavernous hema:
  
  • Large cavernous vascular spaces & dystrophic calcification
  • Found on visceral organs
• Associated w/Von Hippel-Lindau disease
• Hemangioblastoma ^{(tumors of CNS)} in:
  
  • cerebellum
  • eye & bilateral renal cell
  • Cysts in liver/kidney
  • Pheochromytoma

Question 35

Cavernous Lymphangioma

Answer 35

• “Cystic Lymphangioma”
• Common in children in Neck or Axilla
• Defined by size *15 CM in diameter *
• Composed of massively dilated-cystic lymphatic vessels
• Associated w/Turner’s syndrome (45X)
• Causes diff in delivery

Question 36

Bacillary Angiomatosis

Answer 36

• Moist, erosive CUTANEOUS lesion
• _Histo: _
• Acute neutrophilic inflammation
• Vascular (capillary) prolif
• Silver (Warthin-Starry) stain=Tangled bacilli
• Appearance-Black
• Assoc w/Bartonella Henselae ^{(cat-stratch)}

Question 37

Kaposi Sarcoma

Answer 37

• Gross: Red-purple macules & plaques on skin
• Histo: nodular form-sheets of plump proliferating Spindle cells (CD31)
  
  • extravasation of RBCs
• Markers: Vimentin (anchoring)
• Dermal angiosarcoma (malignant neoplasm of endothelial cells)
• Cause = HSV-8 (herpes) affects HIV or immuno-comprimised pts
• Mech: KSHV incorporates in host genome
  
  • Disrupt control of cellular proliferation-inactivating Rb gene
  • Prevents apoptosis of endo cells w/production of tp53 inhibitors (tumor inhibitor)