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Lisa's vasculature has pathology

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Vaso vasorum, where is it most frequently found?
The vasa vasorum consists of a network of arterioles, capillaries and venules, depending on the vessel. They provide the vessel wall that they penetrate with metabolites which they would otherwise not receive due to the thickness of the layers making up the wall. The vasa vasorum is present more frequently in veins than in arteries.
Lues
another name for syphilis
Describe the normal layers of a vessel.
An inner intima, followed by an internal elastic lamina - media - external elastic lamina - adventitia
Where is the most likely area atherosclerosis is found?
Abdominal aorta
Two causes of atherosclerosis:
1. chronic injury to vessel endothelium (ex. caused by hypertension) resulting in chronic inflammation
2. Chlamydia pneumoniae could also cause it
What is an atheroma?
an abnormal inflammatory accumulation of macrophage white blood cells within the walls of arteries
Four components of an atheroma?
1. fibrous cap (smooth muscle cells, dense conn. tissue, macrophages, foam cells)
2. cellular area beneath cap (smooth muscle cells, macrophages, T-lymphocytes)
3. necrotic core (lipid, cholesterol, cellular debris, lipid laden foam cells, Ca)
4. Neovascularization - just peripheral to lesion
What is a fatty streak?
Flat lipid lesion on the vessel wall that includes foam cells and T-lymphocytes that is a precursor to atheromatous plaques found in some infants and all people over the age of 10 y/o
Four complications of atherosclerosis?
1. myocardia ischemia
2. cerebral ischemia
3. peripheral vascular disease
4. aneuryisms
Describe Monckeberg's medial calcific sclerosis: (4)
1. asymptomatic
2. calcific deposits within media of medium sized muscular arteries
3. nonobstructive
4. may be palpated, seen on plain radiographs
What vessels does hypertension induced arteriosclerosis effect?
small arteries and arterioles
There are two types of hypertension induced arteriosclerosis: Hyaline Arteriosclerosis and Hyperplastic Arteriosclerosis. Describe four points of Hyaline arteriosclerosis.
1. pink hyaline thickening of arterioles with lumenal narrowing
2. impairs blood supply of kidney mainly
3. seen in patients with hypertension and diabetes
There are two types of hypertension induced arteriosclerosis: Hyaline Arteriosclerosis and Hyperplastic Arteriosclerosis. Describe hyperplastic arteriosclerosis (2)
1. Onion skin concetric thickening of arteriole wall leading to lumenal narrowing
2. seen in patients with severe acute elevation of BP (malignant hypertension)
Definition of aneurysm:
Localized abnormal dilatation of blood vessel
Describe a true aneurysm:
One where there is no extravasation of blood - the vessel wall is outpounching.
How does atherosclerosis cause an abdominal aortic aneurysm?
It destroys the tunica media and external elastic lamina leading to dilitation of the vessel.
Where is the most common location for a AAA?
Infrarenally (below kidney) and above the aortic bifercation.
How can an AAA be identified during a clinical exam? (3)
1. see a pulsatile abdominal mass
2. may ascultate a bruit (hear swishing of blood)
3. may ascultate a thriss (feel turbulent flow)
What is the size of an AAA that has a large risk of rupture?
Anything over 5cm
What areas does a syphilitic aneuryism effect? (2)
Thoracic aorta and aortic arch
Describe what happens to the blood vessel in syphalitic aneurysm.
"Obliterative endarteritis" of vaso vasorium of aorta:

1. affected vasovasorum have hyperplastic thickening and infiltration by plasma cells and lymphocytes.
2. this kills the tunica media
3. leads to ischemia of aortic wall, weakening it
4. aorta loses elastic recoil forming the aneuryism.
Describe the appearance of the heart in syphilitic aneurysm:
Since it effects the thoracic aorta and aortic arch it also effects the aortic valve. There is a "tree barking" effect on the aorta and aortic valve.
What are the complications of syphilitic aneurysms? (3)
1. aortic valve insufficiency that causes cardiomegaly
2. aortic reguritation
3. compression from the aneurysm onto adjacent structures (airways, lungs, esophagus)
What is an aortic dissection?
an intimal tear of the aorta that allows blood to enter the media of aorta and a blood filled channel is created within the aortic media
What are the two classifications of an aortic thoracic dissection?
Type A (DeBakey Type I and II) - Proximal lesions involving ascending aorta only or ascending and descending

Type B (DeBakey type III)
Distal lesions that do not involve ascending aorta (usually begin distal to subclavian artery)
Two risk factors for an aortic dissection:
1. hypertension
2. Marfan's syndrome
What is the classic presentation of some on with an aortic dissection?
Sudden onset of ripping chest pain radiating to back
Three complications of aortic dissection:
1. rupture
2. retrograde extension toward aorta
3. distal extension away from aorta
What are three treatments of aortic dissections?
1. aggressive BP control
2. surgical intervention
What is the definition of vasculitis:
Inflammation of wall of blood vessels
Where can vasculitis occur?
Anywhere - any vessel in any organ
Symptoms of vasculitis:
"non specific"

fever, malaise, myalgias (muscular pain)
There are two main causes of vasculitis: Direct infection and immunologic causes. Describe the immunologic causes of vasculitis.
In vasculitis Antineutrophil cytoplasmic antibodies (ANCA), which are autoantibodies directed against enzymes found in granules of neutrophils, can form.
VIP

What are the two types of ANCA's (antineutrophil cytoplasmic antibodies) that form in vasculitis?
c-ANCA: has a target of proteinase-3 on the neutrophils. Seen in the cytoplasm when using immunofluoresence. Found in Wegener Granulomatosis.

p-ANCA - has a target of myeloperoxidase. Is found in the perinuclear region of the cell.
What is the most common vasculitis?
Giant cell (temporal) arteritis
What type of vessels are effected in giant cell arteritis?
large vessels - aorta and major branches to head.
What is the mechanism of disease of giant cell arteritis?
A nodular thickening of artery with granuloma formation including giant cells and leukocytes.
What are the symptoms of giant cell arteritis? (6)
headache
facial pain
jaw claudication (pain chewing)
can have blindness and diplopia
muscle weakness
What would an ESR (erythrocyte sedimentation rate) show on a patient with giant cell arteritis?
There would be an elevation
What is the treatment for a patient with giant cell arteritis?
high doses of corticosteriods
What type of vessel is effected in Polyarteritis Nodosa vasculitis?
Medium vessels
What organs arteries are most commonly effected in Polyarteritis Nodosa vasculitis?
Kidney and GI
What vasculature is spared in Polyarteritis Nodosa vasculitis?
Pulmonary (reason unknown)
What does a vessel with Polyarteritis Nodosa vasculitis look like?
It has strictures, aneurysms, necrosis -- the whole wall of the artery is involved.
What age is most suseptable to Giant Cell Arteritis?
over 50 y/o are most likely effected
What age group is usually effected in Polyarteritis Nodosa vasculitis?
People are affected at a young age
What are six signs and symptoms of Polyarteritis Nodosa vasculitis?
Malaise
Fever
Weight loss
renal insufficiency
skin rashes
abdominal pain
In what disease does 30% of patients have Hep B antigen presentation in serum?
Polyarteritis Nodosa vasculitis
What is the treatment of Polyarteritis Nodosa vasculitis?
Immunosuppression
What type vessels does Wegener granulomatosis effect?
Small vessels
What organ's vessels are most often affected in wegener granulomatosis (vasculitis) effect?
Upper respiratory tract
Lung
Kidney
Two potential causes of Wegener Granulomatosis:
1. hypersensitivity to environmental agent or inhaled infectious agent
2. C-ANCA formation
What is present in the serum of 90% of patients with Wegener Granulomatosis?
c-ANCA (anti-neutrophil cytoplasmic antibodies)
What is the "pathology triad" associated with Wegener Granulomatosis?
1. necrotizing granulomas present
2. necrotizing vasculitis present
3. necrotizing glomerulonephritis
What age and sex are most suseptable to Wegener Granulomatosis?
40y/o males
What are 3 symptoms of Wegener Granulomatosis?
1. chronic sinusitis
2. hemoptysis - from mucosal laceration
3. kidney problems
What is the treatment for Wegener Granulomatosis?
immunosuppression
What vessel is most likely affected by Kawasaki Syndrome?
Coronary artery - though any medium vessel can be affected.
What causes Kawasaki Syndrome(vasculitis)?
An unknown virus
What does the virus that causes Kawasaki Syndrome (vasculitis) induce?
Autoantibodies to endothelial and smooth muscle cells causing the vasculitis
What does the immune response caused by Kawasaki Syndrome end up doing to the vessels?
Causes necrosis of the blood vessel wall which can cause aneurysm formation.
What age group is affected by Kawasaki syndrome?
infants and children less than 5 years old
What is "mucocutaneous lymph node syndrome" and what disease causes it?
It is a syndrome associated with:
1. fever
2. conjuctival/oral mucosa erythema (redness)
3. redness in palms and soles
4. skin rash
5. cervical lymph node enlargement

This syndrome is caused by Kawasaki syndrome vasculitis.
What effects to the coronary artery are caused by Kawasaki syndrome?
Coronary artery aneurysms that can rupture causing an MI
What is the treatment for Kawasaki syndrome? (2)
aspirin and immunoglobulins

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