Blood Vessels Flashcards
Mutations in ENaC leading to inc tubular resorption by aldosterone
Liddle syndrome
Mechanisms of Essential Hypertension (4)
1 Reduced renal sodium ex
2 Inc vascular resistance
3 Genetics
4 Environment
Benign hypertension
Homo pink hyaline with thickening of arteriolar wall
Sometimes with nephrosclerosis
Hyaline arteriolosclerosis
Typical severe hypertension
Onionskin concentric laminated thickening and luminal narrowing
Necrotizing arteriolosclerosis
Hyperplastic arteriolosclerosis
Hardening of arteries
Arteriolosclerosis
Calcific deposits in muscular arteries
Older than 50
Do not encroach lumen not clinicially
significant
Monckeberg medial sclerosis
Presence of intimal lesions atheromas
Atherosclerosis
Strongly and independently predict risk of MI stroke PAD SCD even in healthy
CRP level
Atheroma development
Endothelial injury -> endothelial dysfunction and mac and smooth muscle activation ->mac and smooth ms engulf lipid -> smooth ms prolif collagen, ECM, lipid deposition
Minute yellow plaque macules that coalesce into elongated lesions made up of lipid filled foamy mac but do not cause significant flow disturbance
Forms as early as <1 yr
Fatty streak
Most involved vessels in atherosclerotic plaque formation:
Infrarenal abdominal aorta > coronary arteries > popliteal arteries > ICA > circle of Willis
Atherosclerotic plaque 3 principal components:
1 cells (smooth muscle or foam cell, mac and T lymphocytes) 2 ECM (collagen, elastic fibers, proteoglycan) 3 intracellular and extracellular lipid
When plaque exposes highly thrombogenic constituents
Plaque rupture/fissuring
Thrombogenic subendothelial membrane exposed to blood
Erosion/ulceration
Expansion of volume by plaque
Hemorrhage into atheroma
Plaque inflammation induces
collagen degradation
reduced collagen synthesis
True aneurysms
Involve all three layers of artery
False aneurysm
Pseudoaneurysm
Wall defect becoming extravascular hematoma communicating with intravascular space (pulsating)
Pressured blood enters arterial wall through defect and pushes apart underlying layers
Arterial dissection
Discrete outpouchings 5-20cm with thrombus
Saccular
Circumferential dilations up to 20cm involving aortic arch, abd aorta and iliac arteries
Fusiform
Aneurysms occur bec (4)
1 inadequate abnormal CT synthesis
2 excessive CT degradation
3 loss of smooth muscle cell or change in smooth muscle cell synthetic phenotype
Abnormal sequestration of tgf b in wall leading to dilation and dysregulating signalling and loss of elastic tissue
Marfan syndrome
scaffolding protein defective in Marfan
fibrillin
Mutation in tgf b result in
1 defective elastin
2 defective collagen synthesis
Type IV Ehrlos Danlos results fr
Defective Type III collagen synthesis with aneurysm
skin
ligament
blood vessel
Narrowing of aortic vasa vasorum fr sys HTN and ischemia Smooth muscle cell loss Aortic degenerative changes (fibrosis) Inadequate ECM synthesis Inc amorphous proteoglycan
Cystic medial degeneration
Most important causes of aneurysm (2)
1 atherosclerosis
2 hypertension
Factor in abdominal aneurysm
Atherosclerosis
Ascending aortic aneurysms
Hypertension
Embolization of septic embolus fr infective endocarditis
Extension of suppurative process
Direct infection of wall
Mycotic aneurysm
Tertiary syphilis has predilection for
Vasa vasorum of ascending THORACIC aorta
Immune response to spirochetes attacking thoracic aorta
Obliterative endarteritis
Men
Smokers
More than 50
Plaques compromising diffusion of nutrients and waste bet lumen and wall compressing media leading to necrosis and thinning
Abdominal Aortic Aneurysm AAA
Distinct subtype of with dense periaortic fibrosis with lymphoplasmacytic inflammation mac giant cell
Inflammatory AAA
Risk of aneurysm rupture is determined by
Size
Never bursts
AAA =4cm diameter
4-5cm
1% chance rupture
5-6cm
11% per year
> 6cm
25%
AAA Managed surgically
> 5cm
Encroachment of mediastinal structure Cough fr irritation of recurrent Pain by erosion of bone Cardiac from valvular insuff or narrowing of ostia Aortic rupture
Thoracic Aortic Aneurysm
Blood splays apart laminar planes of media forming blood-filled channel within wall
Aortic dissection
Dissecting hematoma reenters lumen of aorta through second distal intimal tear creating second vascular channel
Double barreled aorta
Chronic dissection
Ascending aorta isolated
DeBakey II
Proximal Type A dissection
Ascending aorta with descending aorta
Debakey I
Proximal Type A
Beyond sublavian
Debakey type III
Distal Type B
Sudden excruciating tearing stabbing pain beginning on ant chest radiating between scapula moving downward as tearing progresses
Aortic dissection
Most common cause of death in AD
Rupture of dissection into pericadial, pleural and peritoneal cavity
Pathogenic mechanisms of vasculitis (2)
1 immune mediated
2 direct vascular invasion by pathogen
Mech underlying non infectious vasculitis (4)
1 immune complex
2 antineutrophil cytoplasmic antibodies (ANCA)
3 anti-endothelial cell ab
4 autoreactive T cell
Antibody production assoc in drug hypersensitivity vasculitis (penicillin) infection vasculitis
Immune complex assoc vasculitis
ANCAs (antineutrophilic cytoplasmic antibodies) against enzymes of granules
Diagnostic markers that mirror severity
ANCA vasculitis
Neutrophil azurophilic granule sharing homology of bacterial peptides
Assoc with
Antiproteinase-3
PR3 ANCA
Wegener granulomatosis
Lysosomal granule involving free radical generation
Induced by PTU
assoc with
Anti-myeloperoxidase (MPO-ANCA)
P-ANCA
Microscopic polyangiitis
Churg Strauss syndrome
ANCA Vasculitis are pauci immune bec
They do not contain antibody-complement in lesions
Most common form of vasculitis in elderly
Giant cell Temporal arteritis
Granulomatous inflammation of large to small sized arteries supplying head
Temporal
Vertebral
Opthalmic
Aorta
Giant cell Temporal arteritis
Giant cell arteritis is caused by
T cell mediated immune response to vessel wall antigen
TNF and endothelial cell antibody
MHCII
Responds to steroids
Patchy nodular granulomatous inflamm in inner media on INTERNAL ELASTIC MEMBRANE
multinucleated giant cell
fragmentation of IEL
Giant cell temporal arteritis
Giant cell dx tx
Biopsy histology
Corticosteroids TNF
granulomatous vasculitis of medium and large arteries with transmural scarring and thickening of aorta (pulseless)
Ocular disurbance
Weakening of pulse in UE
Less than 50, autoimmune etiology
Takayasu arteritis
Takayasu classically affects
aortic arch and arch vessel
50% pulmonary artery, renal coronary
Systemic vasculitis of small and medium muscular artery involving renal and visceral vessel sparing pulmonary circulation
Assoc with chronic hep B
Segmental transmural necrosis, inflammation and thrombosis
Kidney heart liver
PAN
Acute phase transmural inflammation
infiltrate neutrophils and mononuclear cells accompanied by
All stages of activity in PAN COEXIST
Fibrinoid necrosis and thrombosis
Rapidly accelerating HTN with renal involvement
Abdominal pain bloody stool
Diffuse muscular ache and pain
Peripheral neuritis of motor nerves
Renal major cause of death
PAN
Arteritis of large and medium vessel with involvement of coronary artery causing aneurysms and MI
Infectious trigger
Delayed type hypersensitivity
Cytokine and B cell activation
Kawasaki Disease
Transmural inflammation with less fibrinoid necrosis and accompanying aneurysm
Kawasaki
Conjunctival oral erythema Blistering edema of hands Erythema of palms and soles Desquamative rash Cervical LN enlargement (mucocutaneous LN) CV sequelae
Kawasaki
Necrotizing vasculitis affecting capillaries, small arterioles and venules characterized by segmental fibrinoid necrosis of media with focal transmural necrosis lesion
Also called hypersensitivity or leukocytoclastic vasculitis
Microscopic polyangitis
All lesions of the same age
Pauci immune
90% nec glomerulonephritis Pulmonary capilitis Hemoptysis Hematuria Proteinuria Abd pain Bleeding
Microscopic polyangitis
Wegener Granulomatosis triad:
Granuloma of lung URT
Vasculitis small to med in lungs
Glomerulonephritis
PR3 ANCA +
Like PAN but with pulmo sx
Nec granulomatous vasc with surrounding fibroblast prolif
MAN 40 yrs with bilateral pneumonitis nodule and cavitary lesion, mucosal ulcer, renal disease
Mort of 80% 1 yr
Wegener Granulomatosis
Wegener tx
Rituximab anti b cell
cyclophosphamide
Steroid
Allergic granulomatosis and angiitis
Small vessel necrosis assoc with asthma, allergic rhinitis, lung infiltrate and eosinophil perivascular infiltration
MPO ANCA+
Granuloma and eosnophil
Churg Strauss syndrome
Heavy smoker Before 35yrs Cold induced Raynaud phenomenon Instep claudication Superficial nodular phlebitis (venous inflamm) Gangrene of extremities
Sharply segmental acute and chronic transmural vasc of medium sized and small arteries with microabscess
Thromboangiitis obliterans
Buerger disease
Ischemic dilated myopathy assoc with emotional duress from cardiac raynaud
Takotsubo cardiomyopathy
Broken heart syndrome
Evanescent thrombosis in diff vascular beds at diff times from hypercoagulable states
Trousseau syndrome
Migratory thrombophlebitis
Pancreatic cancer
DVT risk factors
CHF pregnancy OCP Obesity Malignancy Post op immob
Common serious clinical complication of DVT
Pulmonary embolism
First manifestation of thrombophlebitis
Pulmonary embolus
Neoplasm bronchogenic ca or mediastinal lymphoma obstructing SVC
Dilation of veins of head neck arms and lungs with cyanosis and respi distress
SVC syndrome
IVC result from
Compression or invasion of IVC by renal or HCC
Familial disease of agenesis or hypoplasia of lymphatics
Milroy
Heredofamilial congenital lymphedema
Permanent dilation of preexisting small vessels in skin and mucous membrane forming discrete red lesions
Telangectasia
Most common form of vascular ectasia
Nevus flammeus birthmark
Lesions on childhood thickening skin surface and do not fade with time occuring on distribution of trigeminal nerve assoc w Sturge Webber (encephalotrigeminal angiomatosis)
Port wine stain
AD by mutation of genes encoding TGF B
Dilated cap and veins and rupture
Hereditary hemorrhagic telangiectasia (Osler Weber Rendu disease)
Cavernous hemangiomas constitute a component of
von Hippel Lindau disease
vasc lesions on cerebellum brainstem retina pancreas liver
Benign painful tumors from smooth muscle cells of glomus bodies involved in thermoregulation
Glomus tumors
Vascular proliferation in immunocompromised hosts caused by G- of Bartonella family
Cats
Homeless
Bacillary angiomatosis
vascular neoplasm by KSHV or HHV8
most common in AIDS
Kaposi sarcoma