Cardiovascular Path 8 Flashcards
microscopic polyangitis is associated with ____ ANCA
P-ANCA (MPO-ANCA)
will see fragmentation of WBC’s (neutrophils)
histologically, microscopic polyangitis looks like ____ with:
PAN: segmental fibrinoid necrosis of media with focal transmural necrotizing lesions but WILL NOT SEE INFARCTION like we see in PAN. can see this in the capillaries which you won’t see in PAN
NO GRANULOMAS
is there a granulomatous response in microscopic polyangitis
NO, but if it did, we would diagnose it was Wegener’s granulomatosis ; however it does affect the pulmonary vessels
what are some differences between PAN and microscopic polyangitis?
PAN: involves infarcts while microscopic polyangitis does not
PAN DOES NOT involve the lungs but microscopic polyangitis does
all lesions are the same/different age in microscopic polyangitis
same; homogenous
what are some causes of microscopic polyangiitis
- HS to drugs: penicillin
- HS to infections (strep)
- positive for MPO-ANCA
No IgG: pauci immune
wegener’s granulomatosis = _______ + ________
microscopic polyangitis + granulomatosis
what is the classic triad of features seen in Wegener’s granulomatosis
- acute necrotizing granulomas of upper and lower respiratory tract
- focal necrotizing or granulomatous vasculitis affecting small to medium vessels
- renal disease in the form of focal or necrotizing, often crescentic glomerulonephritis
microscopic polyangitis can affect any subset of vessels but in Wegener’s _____, _____, and _____ are most commonly involved
upper and lower respiratory tract and renal involvement
95% of patients with Wegener’s have ______ ANCA
PR3/C-ANCA
what are some common clinical features of Wegener’s granulomatosis?
think upper, lower respiratory and renal:
- persistant pneumonitis with bilateral nodular and cavitary infiltrates (HEMOPTYSIS)
- chronic sinusitis
- mucosal ulceration of nasopharynx
- evidence of renal disease such as hematuria, renal failure ,etc
patient comes in with sinusitis symptoms and upon PE you find ulcerative lesions of the nose, pharynx and palate. what is this disease?
Wegener’s
lesions in wegener’s ultimately undergo _______
progressive fibrosis and organization
Churgg Strauss syndrome is also called ______
eosinophilic granulomatosis with polyangiitis
Churg Strauss has positivity for _____ ANCA
P-ANCA
_____ syndrome is characterized by granulomas, transmural necrosis and eosinophilic infiltration
Churg Strauss
patients with Churg Strauss usually are associated with _______, ______ or ______
- allergic rhinitis
- bronchial asthma
(they have eosinophilia so think type 1 HS) - peripheral neuropathy (wrist/foot drop)
- skin nodules or purpura
how can you tell the difference between cavitations in the lung due to tb and Wegener?
- NO acid fast bacilli in Wegners
- inflammation in more closer to vessels in Wegener’s
lesions in Wegener’s strauss ultimately undergo _______
progressive fibrosis and organization
allergic + wegener’s (granulomatosis and angiitis) =
Churg Strauss
patients with _______ (vasculitis) can present with hematuria
Granulomatosis with polyangitis (Wegener’s)
what is an autoimmune disease where patients can have both renal and lung involvement?
Goodpastures
______ and _____ can lead to RPGN and see crescents in the glomerulus
Wegener’s and Goodpastures;
differentiate with immunofluorescence
describe Churg Strauss disease in 3 words
allergic granulomatosis w/ angiitis
how can aneurysm be classified?
based on:
- composition of the wall: true vs false aneurysm
- gross morphology: saccular vs. fusiform
- etiology: atherosclerosis, ANCA, HTN, etc
what is a false / pseudo aneurysm?
an extravascular hematoma that communicates with the intravascular space;
what is the difference between fusiform and saccular type of aneurysm
fusiform: BOTH sides of the vessel is dilated
saccular: only one side is dilated
What are some common causes of aneurysms (6)
- atherosclerotic
- syphilis
- infections (mycotic)
- vasculitic (PAN, Kawasaki’s)
- congenital: Marfan’s / Berry aneurysm
- iatrogenic: aneurysm in chronic renal failure patients on dialysis
atherosclerosis can cause aneurysm by ________ of the tunica media
pressure atrophy → weakening of the media
how does syphillis cause aneurysm?
- can lead to end arteritis obliterans and can cause blockage of the vasa vasorum → fibrosis and blood vessels loses strength
most common site of atherosclerotic aneurysm is the _______
abdominal aorta
the MMP’s (matrix metaaloproteinases) produce day the macrophages in ______ can cause:
atherosclerosis
- eat up the fibrin cap and lead to an unstable plaque
- degrade components of the ECM in the arterial wall: collagen, elastin, proteoglycans, laminin and fibronectin → weakened media → chance for aneurysm
damage and weakening of the ECM in atherosclerotic aneurysm can be due to: (2)
- matrix metalloproteinases released by macrophages
- deficiency of tissue inhibitor of proteinases (↑ ↑ proteinases)
abdominal aneurysms can impinge on adjacent structures such as _______ or _____.
ureter or erosion of vertebrae
secondary infection by _______ in someone with an abdominal aneurysm will ↑ the chances for rupture
Salmonella
syphilitic aneurysm usually affects the ______
thoracic aorta (ascending aorta)
inflammatory response to the spirochetes in syphilis will cause _________ of the vaso vasorum that supplies the ________ leading to ____ injury of the tunica media of the aorta
obliterative endarteritis;
aorta;
ischemic injury (due to narrowing of the lumen)
“tree bark” appearance is seen in _______ due to the fibrosis of the vascular wall
syphilitic aneurysm
wrinking of the aortic_____ due to secondary atheroslceroiss can cause ____
intima; narrowing or occlusion of coronary ostea
_______ can occur in syphilitc aneurysm if the lesions extend downward
aortic valve insufficiency
cor bovium (cow’s heart) is a potential complication of ________ due to _____
syphilitc aneurysm; it can potentially cause aortic valve insufficiency → aortic regurgitation → massive LVH
what are some clinical symptoms of someone suffering from syphilitic aneurysm?
occurs at the ascending aorta so it can encroach mediastinal structures:
- respiratory diffuculties (bronchus)
- difficulty swallowing
- persistent cough due to recurrent laryngeal nerve compression
- pain
what is the most common cause of death in patients with syphilic aneurysm
CHF due to LVH
potential complications of syphilitic aneurysm
- CHF (most common COD)
- cardiac ischemia
- rupture
Marfan’s syndrome is a defect in the gene for ______ which is required for ______
fibrillin - 1 which is required for normal elastic tissue development
describe the physical features of someone with Marfan’s
- elongated axial bones, tall and slender
- long thin extremities and fingers
- subluxation of the lens because ciliary body is rich in fibrillin
cystic medial degernation of the ECM is seen in patients with ______
Marfan’s, other diseases that cause abnormal ECM, and severe HTN ; ECM does not have elastic fibers so there is mucoid material instead leading to cystic medial degeneration
berry aneurysm is a developmental ______ walled aneurysm in the circle of willis
thin walled; small and saccular
rupture in the circle of willis in berry aneurysm can occur during _____ or _____ and can lead to a ______ hemorrhage
orgasm or straining at stool;
subarachnoid hemorrhage → headache and coma
What is an aortic dissection?
it is when there is a tear in the intima layer of the vessel which then leads to blood seeping out and moving along the laminar planes of the media and separating the two layers
(extravasation of blood)
not usually associated with marked dilation of the aorta
in older people, those with ______ usually suffer form aortic dissection
HTN
double barrel aorta is a complication of ______
aortic dissection
What are some causes for aortic dissection? (4)
- HTN
- connective tissue disorders: Marfan’s and Ehler-Dnanlos Syndrome
- complication of arterial cannulation
- pregnancy induced
_________ changes and __________ are both ways that HTN can cause aortic dissection
ECM degernative changes and variable loss of medial smooth muscle cells;
(ischemic and pressure plays a role)
_____ type of aortic dissection has the worse prognosis
proximal; because it involves the ascending aorta → progress further and involve the pericardium → fatal hemorrhage and cardiac tamponade OR
what are complications of the proximal type (type A) of aortic dissection:
- cardiac tamponade: the ascending aorta is involved in the proximal type and so it can progress further to involve the pericardium → hemorrhage → cardiac tamponade
- MI: retrograde dissection into the aortic root leading to disruption of aortic valve or coronary artery
HIGH MORTALITY
Type B (distal) aortic dissection involves the _______
descending aorta to the left subclavian artery so can see renal artery involvement
sudden onset of chest pain that radiates to the back and is felt between the scapulae and moves down is a common clinical presentation of someone with ______
aortic dissection
what are some clinical features of aortic dissection?
- chest pain that radiates to the back and is felt between the scapulae
- loss of one or more arterial pulses
in an aortic dissection rupture into the lumen leads to ______ and external rupture leads to ______
double barrel aorta;
cardiac tamponade
what will you seen on histology of someone with aortic dissection:
- cystic medial degernation: focal lass of elastic and muscle fibers in the media which are filled with myxoid material
NO INFLAMMATION
can someone with atherosclerosis develop aortic dissection?
NO because with atherosclerosis there is an inflammatory response that leads to fibrosis of the vessel wall → protective against aortic dissection
what are some possible complications of aortic dissection?
- extension into renal, mesenteric or iliac arteries → critical obstruction
- compression of spinal arteries → transverse myelitis *(paresethesia and weakness in legs and arms)
- rupture
- MI
- cardiac tamponade
