peripheral vascular disease

Question 1

Aneurysm (congenital or Acquired)

Answer 1

Localized dilatation of BVs (aorta or heart

Types: True (involves all three layers of wall), False or pseudo aneurysms- wall defect leading to extravascular hematoma

Arterial dissection- blood enters the wall of the artery

Question 2

Pathogenesis of aneurysms

Answer 2

Factors affecting collagen structure or function: inadequate or abnormal syntheses of collagen (marfans - defective fibrillin synthesis, no scaffolding of elastic tissue, results in cystic medial necrosis of aorta, leads to aneurysm formation and aortic dissection

Ehlers Danlos syndrome: one variant has defective type 3 collagen, leads to aneurysm formation

Excessive CT degradation (increased MMPs or decreased TIMPs that inhibit the MMP), inflammation (atherosclerosis) polymorphisms of MMP or TIMP may predispose to aneurysm formation

Loss of smooth muscle cells–thickening of intima (due to atherosclerosis)- leads to ischemia or inner media, systemic hypertension, (narrows vasa vasorum–ishcemia of outer media), morphology results in cystic medial degeneration

Question 3

Specific underlying causes of aneurysms

Answer 3

Common: atherosclerosis- abdominal aorta
Hypertension- ascending aorta

Uncommon- congenital defects (berry aneurysm- bifurcation of cerebral areteris, subarachnoid hemorrhage) infections (mycotic aneurysm- septic emboli, direct extension, direct infection via circulating organisms, syphillis (treponema pallidum) trauma- AV fistula aneurysm, vasculitis, igG4 disease, genetic defects in collagen (marfans and ehlers Danlos

Question 4

Aortic aneurysms

Answer 4

Locations- abdominal aortic aneurysm (AAA) most common location, below renal arteries and above bifurcation

Arch of aorta, thoracic aorta, and iliac arteries less common

PAthogenesis/etilogy- men smokers, atherosclerosis

Morphology- up to 15 cm in diameter, thinning and destruction of media, mural thrombus, saccular or fusiform, variants (inflammatory- unknown etiologu, mycotic (secondary to infection (salmonella)

Question 5

Abdominal aortic aneurysm clinical course

Answer 5

more common in men over 50, present as pulsatile abdominal mass, complications (rupture into peritoneum, retroperitoneum). vascular obstruction (renal mesenteric spinal arteries, embolism of atheroma or mural thrombus to kidneys or lower extremities, impingement of ureters

Aggressive management for large 5 cm aneurysms risk of rupture is directly proportional to size

Question 6

Thoracic aortic aneurysms

Answer 6

Hypertension, Marfans syndrome, Syphilis (tertiary)

Clinical features: Mediastinum encroachment, tracheal compression, esophageal compression, bone erosion cough due to irritation of recurrent laryngeal nerve

Cardiac symptoms: heart failure due to aortic valve insufficiency

Aortic rupture

Question 7

aortic dissection

Answer 7

Blood between and along laminar planes of media- causes a blood-filled channel that easily ruptures

Pathogenesis/etilogy- HTN (40-60 yrs of age), >90% of cases

CT abnormalities (younger ages): in association with Marfans syndrome or Ehlers-Danlos syndrome

Ascending aorta most commonly involved
Rare causes: post procedural- arterial cannulation, pregnancy

Question 8

Morphology of Aortic dissection

Answer 8

intimal tear within Cm of aortic valve, dissection plane between middle and outer third of wall , in media and

Dissection usually extends anterograde, usually ruptures outs, may reenter and form a double barreled lumen

In HTN: vasa vasorum hyaline arteriolosclerosis, media loss of smooth muscle cells

Pre existing pathology of cystic medial degeneration in patients with CT disorders.

Question 9

Aortic dissection: clinical course

Answer 9

dependent on level of aorta involved, sharp paon of anterior chest, uneven pulses and widened mediastinum

Type a- both sides, type b one side

Classic clinical symptoms- sudden onset of tearing or stabbing pain in anterior chest radiating to back

Question 10

Complications of aortic dissection

Answer 10

Hemopericardium occurs when blood dissects thru media, proximally

May also rupture into pleural or peritoneal cavities

Question 11

Vasculitis

Answer 11

inflammation of vessel walls, may affect any sized vessel, pathogenesis (immune-mediated processes, infectious origin, unknown)

Question 12

path of immune mediated vasculitis

Answer 12

Immune complex mediated: SLE (DNA anti DNA complexes), hypersensitivity to drugs, viral infections (HBsAg-antiHBSsAg)

ANCA associated: auto antibodies against enzymes in granules of PMNs Ab titers mirror clinical severity
2 main patterns: Antiproteinase-3 (PR3-ANCA)–granulomatosis with polyangiitis and Chirg-Strauss syndrome (

Question 13

Vasculitis

Answer 13

infectious (direct invasion- syphilis, aspergillus, mucormycosis)
Indirect via immune mechanisms triggering cross-reactivity

Unknown Giant cell arteritis, takayasu arteritis, polyarteritis nodosa

Question 14

Giant cell (temporal) arteritis

Answer 14

Most common form of systemic vasculitis in adults–affects aorta and its major branches, especially temporal artery, ophalmic, and vertebral arteries

Pathogenesis: uncertain, T cell mediated driven by antigen

> 50y, often with painful superficial temporal artery, diplopia, visual loss, headache

increased ESR, diagnosis (biopsy) treat with steroids

Gross: nodular thickenining of artery with narrowed lumen, patchy (discontinuous segments affected, biopsy requires 2-3 cm
histo: granulomatous inflammation of inner half of media around internal elastic lamina (destroys internal elastic lamina), multinucleated giant cells

Question 15

takyayasu arteritis

Answer 15

pulseless disease (upper extremities, ocular disturbances, jap fem under 40
granulomatous inflammation of aortic arch and its branches
pulm arteries involve half of time, coronary and renal arteries sometimes involved
pathogenesis-autoimmune etiology, histo (lymphocytes giant cells, collagenous fibrosis)

Question 16

polyarteritis nodosa classic

Answer 16

systemic segmental transmural necrotizing inflammation of small/med sized muscular arteries

Renal and visceral arteris affected, usually spares lungs

Focal, random, episodic, all stages of inflammation and fibrosis co exist, aneurysms, young adults (30% Hep B ag), no ANCA
Acute chronic subacute or remittent course
90% remission or cure (corticosteroids, cyclophosphamide)
Classic presentation: Rapidly accelerating HTN, abdominal pain, bloody stools, peripheral neuritis

Question 17

kawasaki syndrome

Answer 17

Endemic in Japan
Large med and small arteries, often coronaries with aneurysm formation
With mucocutaneous lymph node syndrom (mucous membrane inflammation, enlarged lymph nodes)

80% are under 4 yrs, leading cause of acquired heart disease in kids in japan
Histo: necrosis and inflammation, aneurysms, resembles polyarteritis nodosa
Etiology: Ab and viral trigger

Question 18

Microscopic polyangiitis (leukocytoclastic vasculitis

Answer 18

Arterioles, capillaries, venules
Skin mucous membranes, lungs, brain, heart, GIT , kidneys, muscles (necrotizing glomerulonephriteis 90%, and pulm capillaritis)
MPO ANCA in 70%
Immunologic rxn to Ag (drug, Strp, tumor proteins)
histo: fibrinoid necrosis, neutrophils, nucleat dust
Major clinical features are dependent on vascular bed involved: hemoptysis, hematuria, proteinuria, abdominal pain or bleeding muscle pain or bleeding and cutaneous purpura

Question 19

Granulomatosis with polyangiitis

Answer 19

Necrotizing vasculitis (granulomas of lung ndor upper respiratory tract –ear, nose, sinuses, throat)
Vasculitis of small to medium sized vessels in lungs and upper respiratory tract
Glomerulonephritis

PR3-ANCA present in 95%, cell mediated hypersensitivity responses against inhaled infections or environmental Ag

M>F (40 yrs of age) , Symptoms (pneumonitis, sinusitis, nasopharyngeal ulceration, renal

Question 20

Churg Strauss Syndrome

Answer 20

Small vessel vasculitis associated with (asthma, allergic rhitis, lung infiltrates, peripheral eosinophilia and infiltration of vessels by eosinophils, extravascular necrotizing granulomas)

Clinical symptoms: palpable purpura, GI bleed, Renal impairment, cardiomyopathy

Question 21

Thromboangiitis obliterans beurgrers disease

Answer 21

inflammation and thrombosis of med to small musc arteries and secondarily the veins and nerves, stibial and radial arteries, smokers, painful ischemic disease, gangrene of limbs requiring amputation, etiology unknown

Question 22

Raynauds phenomenon

Answer 22

Paroxysmal pallor or cyanosis of fingers, toes, nose, or ears
primary- recurrent vasospasm , young healthy women,
Sec- arterial insufficiency- narrowing SLE SS atherosclerosis bergerus disease

Question 23

Varicose veins

Answer 23

Varicosity- venous dilation due to elevated pressure and or weakend vessel wall (dilation lead to valvular incompetence, edema, pain, thrombosis, stasis dermatitis and ulcers
Obesisty, pregnancy
Superficial leg and veins (low risk of embolism)
Esophageal varices- caused by portal HTN form cirrhosis or portal vein thrombosis, susceptible to rupture and bleeding
Hemorrhoids (anorectal veins, from preg and strain)