Vascular Pathology II Flashcards
what are the three types of arteriosclerosis?
- arteriolosclerosis 2. atherosclerosis 3. Monckeberg medial sclerosis
what is monckeberg medial sclerosis?
age related degenerative calcific change where calcium deposits are found within the media of the artery; very little clinical significance
what is the inheritance pattern in most genetic cases of atherosclerosis?
multifactorial inheritance
what effect does estrogen have on atherosclerosis?
a protective effect
what is key to the drive of pathogenesis of atherosclerosis?
the endothelium becomes activated
what occurs during smooth muscle recruitment due to endothelial activation?
smooth muscle cells migrate from the media of the vessel to the intima and synthesize extracellular matrix. end result: intimal thickening
what 3 factors cause smooth muscle proliferation and extracellular matrix deposition?
PDGF, fibroblast growth factor, an TGF-alpha
what is the difference between a saccular aneurysm and a fusiform aneurysm?
saccular bulges out on one side and fusiform is on both sides
what is the difference between a true and false aneurysm?
a true aneurysm has an intact thinned muscular wall; false aneurysm has a defect through the wall
what is cystic medial degeneration and what can it lead to?
the process where the normal elastin of the media is disrupted and replaced with areas of amorphous ground substance. It can cause aneurysm and dissection
when might you see cystic medial degeneration?
Marfan syndrome, Ehlers Danlos syndrome, and systemic hypertension
what mutation leads to marfan syndrome?
FBN1 fibrillin gene
what occurs in marfan syndrome?
defective synthesis in fibrillin leads to its inability to bind TGF-beta. the increased TGF-beta activity causes weakening of the elastic tissue, predisposing to aortic aneurysm and aortic dissection
what mutation leads to Ehlers Danlos syndrome?
mutation in collagen
which form of Ehlers danlos syndrome predisposes the patient to arterial rupture of large arteries?
the vascular form, which is caused by a mutation in collagen 3
what is the most important risk factor for AAA?
atherosclerosis
where is the typical location for an AAA?
below the renal arteries, above the aortic bifurcation
5-10% of aortic aneurysms are of inflammatory type with abundant lymphoplasmacytic inflammation. A subset of these are caused by IgG4 related disease. What does this mean?
it is a chronic inflammatory disease= numerous lymphocytes and IgG4 secreting plasma cells associated with prominent fibrosis
what is the clinical relevance of IgG4 disease and AAA?
it responds well to steroids
what is the most important risk factor for thoracic aortic aneurysm?
hypertension
besides HTN, what are the other causes of thoracic aortic aneurysm?
untreated syphillis, connective tissue diseases, and vasculitis
when does tertiary syphilis present?
after a latency period of 5 years or more after the initial infection
what cardiovascular effects can syphilis cause?
obliterative endarteritis of vasa vasorum–> thoracic aneurysm; aortitis; aortic regurgitation
aortic dissection has a classic clinical triad of what?
1) abrupt onset of thoracic pain with a sharp, tearing or ripping character 2) variation of pulse (absence of proximal extremity or carotid pulse) and a greater than 20 point blood pressure difference between arms 3) mediastinal widening on chest x-ray as blood expands the vessel
what are the two main causes of aortic dissection?
hypertension and connective tissue disorders
what does biopsy of the temporal artery show in a patient with GCA?
intimal thickening and granulomatous inflammation with fragmentation of internal elastic lamina
what are the signs and symptoms of takayasu arteritis?
nonspecific, but ocular disturbances and weakened pulses in the upper extremity are often present
what is the classic presentation of polyarteritis nodosa (PAN)?
rapidly accelerating HTN, abdominal pain and bloody stools, myalgias, peripheral neuritis
what does histology show in a patient with PAN?
necrotizing (fibrinoid necrosis) with mixed segmental inflammation
how do you treat PAN?
immunosuppression
what is kawasaki disease caused by?
activated T cells which are thought to be provoked by viral infectious illnesses
what is churg-strauss syndrome associated with?
asthma, hypereosinophilia, lung infiltrates
what is the pathogenesis of GPA (Wegener’s granulomatosis)?
T cell hypersensitivity reaction thought to be mediated by inhaled environmental or microbial material
what do the lung biopsies show in a patient with wegener granulomatosis?
cavitary necrotizing granulomatous inflammation
what do kidney biopsies show in a patient with wegener’s granulomatosis?
focal necrotizing and crescentric glomerulonephritis
what is the prognosis of GPA (wegener’s granulomatosis)?
left untreated, GPA is rapidly fatal
how do you treat wegener’s granulomatosis?
steroids, cyclophosphamide and TNF-antagonists
which vasculitides affect the aorta?
GCA and takayasu
what vasculitides has neutrophils present?
behcet’s disease
what vasculitides has eosinophils present?
churg-strauss syndrome
which vasculitis must have a granuloma present?
wegener’s granulomatosis
what is the serum ANCA for wegener?
pr-3 ANCA
what is the serum ANCA for churg strauss syndrome?
MPO ANCA
what is the serum ANCA for microscopic polyangiitis?
MPO ANCA
what are two types of coronary intervention?
balloon angioplasty and endovascular stenting
what is the solution to restenosis following balloon angioplasty and endovascular stenting?
drug eluting stent
which vein graft has the longer patency?
internal mammary artery
