Vascular Path Flashcards
What are the three layers of vessels, from inside to out?
Intima
Media
Adventitia
What layer of vessels houses the muscular layer?
Media
True or false: most cases of vasculitis are infectious in origin
False–usually idiopathic
What are the clinical features of vasculitis?
- Nonspecific ssx of inflammation
- Symptoms of organ ischemia
How do the ssx of organ ischemia arise in the setting of vasculitis? (2 ways)
Thrombosis at the site of BM/collagen exposure
Fibrosis secondary to healing narrows the lumen
What vessels do the large vessel vasculitides affect?
Vasculitis involving the aorta or its major branches
What is temporal (giant cell) arteritis?
Vasculitis of the branches of the carotid artery (usually temporal artery)
Who usually gets temporal arteritis?
Usually females over the age of 50
What are the ssx of temporal (giant cell) arteritis?
- HA
- Visual disturbances
- jaw claudication
- Polymyalgia rheumatica
What labs are classically elevated in temporal arteritis?
- ESR (usually greater than 100)
- CRP
- Platelets
What are the histological characteristics of giant cell vasculitis?
Inflamed vessel wall with giant cells, intimal fibrosis, and granulomatous vasculitis
Why do you need to take a large biopsy of an artery if you suspect temporal (giant cell) arteritis? Can this biopsy exclude the disease?
Can affect isolated parts of the artery, thus a single biopsy will not exclude the disease
What is the treatment for temporal (giant cell) arteritis? What is the major sequelae of this if left untreated?
Corticosteroid to reduce inflammation
High risk of blindness d/t ophthalmic artery involvement
True or false: if you suspect temporal (giant cell) arteritis, you should treat immediately, even without confirmation
True
What is Takayasu arteritis? Where in the large arteries does it usually occur? In whom does it usually present?
Granulomatous vasculitis similar to temporal arteritis, but involving vessels at the branch points of the aortic arch
Classically presents in asian females younger than 50 yo
What are the ssx of Takayasu arteritis? (2)
- Visual and neurological ssx
- Weak or absent pulse in an UE (“pulseless disease”)
What lab is classically elevated in Takayasu arteritis?
ESR
What is the treatment for Takayasu arteritis?
Corticosteroids
What are the major differences between temporal arteritis and Takayasu arteritis?
Temporal arteritis usually affects branches of the carotid artery, whereas Takayasu’s is aortic branches
Which vessels are affected in medium-vessel vasculitis?
Muscular arteries that supply the organs
What is polyarteritis nodosa? What organ is spared?
necrotizing vasculitis that can affect a variety of vessels/organs, but
-spares the lungs
Who usually gets polyarteritis nodosa? S/sx?
- Young adults
- Ssx depends on organ/vessel involvement, but spares the lungs
What serum marker is usually elevated in polyarteritis nodosa?
HBsAg
What are the histological characteristics of polyarteritis nodosa? Imaging finding?
Transmural fibrinoid necrosis that heals, resulting in nodes of fibrous tissue
“string-of-pearls” appearance on imaging
What causes the “string-of-pearls” appearance on imaging with polyarteritis nodosa?
Areas of fibrinoid necrosis/fibrotic healed areas, interspersed between weakened areas characterized by aneurysms
What is the treatment for polyarteritis nodosa? What happens if it is left untreated?
Corticosteroids and/or cyclophosphamide
Fatal if not treated
What is Kawasaki’s disease? Who does it usually affect?
Vasculitis that classically affects children under 4 yo
What are the s/sx of Kawasaki’s disease? (4)
- Fever
- Conjunctivitis
- Erythematous rash of the palms and soles
- Cervical LAD
What is the preferential artery that is involved with Kawasaki’s disease? What are the potential consequences of untreated Kawasaki’s disease?
- Coronary
- Thrombosis with MI
- Aneurysm with rupture
What is the treatment for Kawasaki’s disease? Prognosis?
- ASA and IVIG
- Disease is self-limited but need to prevent severe sequelae
What is the only illness where ASA is appropriate for use in children? Why?
Kawasaki’s disease
Prevents platelet COX and TXA2 formation on sites of collagen exposure
What is Buerger Disease?
necrotizing vasculitis involving the digits, that presents with ulceration, gangrene, and autoamputation of fingers and toes
What is the common phenomenon that is present with Buerger’s disease?
Raynaud
What is Buerger’s disease highly associated with?
Smoking
What are the vessels that are affected in small-vessel vasculitis?
- Arterioles
- Capillaries
- Venules
What is Wegener’s granulomatosis?
Necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and kidneys
What are the three organs that are involved in Wegener Granulomatosis? (“C” disease)
- Nasopharynx
- Lungs
- Kidneys
What is the classic Ab that is elevated in Wegener’s granulomatosis? What is the significance of this relative to disease activity?
- C-ANCA
- Correlates with disease activity
What is the treatment for Wegener’s granulomatosis?
- Cyclophosphamide
- Corticosteroids
What are the presenting ssx of Wegener’s granulomatosis? (3)
- Sinusitis or nasopharyngeal ulcerations
- Hemoptysis with bilateral nodular involvement
- Hematuria d/t RPGN
Who usually gets Wegener’s granulomatosis? What kidney pathology do they classically experience?
Middle aged males
Rapidly progressive glomerulonephritis
What is the difference between pANCA and cANCA?
pANCA = perinuclear ab rxn cANCA = centromeric ab rxn
What are the biopsy findings with Wegener’s granulomatosis?
Large necrotizing granulomas with adjacent necrotizing vasculitis and giant cell
What is microscopic polyangiitis?
Necrotizing vasculitis involving multiple organs, especially the lungs and the kidneys
What are the primary differences between microscopic polyangiitis and WG?
nasopharyngeal and granulomas are absent in microscopic polyangiitis, and p-ANCA is found with MPA
What abs correlate with microscopic polyangiitis?
pANCA
What is the treatment for microscopic polyangiitis?
Corticosteroids and cyclophosphamide
What is Churg-Strauss syndrome? What organs are classically affected?
Necrotizing vasculitis with eosinophils that usually involves lungs and heart
What ab levels correlate with Churg-Strauss syndrome?
pANCA
What are the two vasculitides that cause elevated pANCA? How do you differentiate between the two? (3)
- Microscopic polyangiitis
- Churg-Strauss syndrome
-Granulomas, asthma, and peripheral eosinophilia seen in Churg-Strauss
What is the cause of Henoch-Schonlein-Purpura (HSP)?
Vasculitis d/t immune IgA complex deposition
What is the most common vasculitis in kids?
HSP
What are the ssx of HSP? (3)
- Palpable purpura on buttocks and legs
- GI pain/bleeding
- Hematuria
What is the common history of HSP? Why?
Usually occurs following an URI since the respiratory system produces large amount of IgA
What causes the palpable purpura with HSP?
inflammation around IgA deposition
What causes the hematuria found in HSP?
IgA nephropathy
What is the treatment and prognosis for HSP?
**Usually self limited but may recur
Treat with steroids if severe**
What is systemic HTN defined as?
Greater than 140 / 90
SBP is a function of what? DBP?
SBP = f(SV) DBP = f(TPR)
Does Na intake have an effect on SBP, DBP, or both?
Both
What is secondary HTN? What percent of HTN cases are caused by a secondary condition?
5% of cases
How does renal artery stenosis cause systemic HTN?
Increases plasma renin, which converts angiotensinogen to ANG I
What are the two ways that ANG II raises the BP?
- Contracts arteriolar smooth muscle
- Promotes aldosterone release (increases Na reuptake, dumps K)
What part of the nephron does aldosterone act on?
DCT
From where is aldosterone synthesized?
Zona glomerulosa of adrenal gland
What will happen to a kidney in unilateral renal artery stenosis?
Atrophy of affected kidney
What are the two major causes of renal artery stenosis?
- Atherosclerosis
- Fibromuscular dysplasia
What is fibromuscular dysplasia? In whom is it commonly seen?
- Developmental defect of the blood vessel wall that results in thickening of large to medium sized arteries, especially the renal artery
- Young females
What is benign HTN?
Mild or moderate elevation in BP that is clinically silent
This causes organ and vessel damage over time
What is malignant HTN?
Severe elevation in BP (greater than 200/120)
Causes end organ damage, and is a medical emergency
What are the two ways that malignant HTN can arise?
**De novo
From preexisting HTN**
Which part of the vessel wall becomes thick with atherosclerosis? In what size vessels does this usually occur in?
- Tunica intima
- Medium to large sized vessels
What is arteriolosclerosis? What sized vessels does this affect?
Deposition of protein or increase in smooth muscles of the wall in small sized vessels
What is Monckeberg’s medial sclerosis?
Thickening of the media of the vessel
What, generally, is atherosclerosis?
Intimal plaque that obstructs blood flow
What are the components of an atheroma?
Lipid core with foam cells surrounded by a fibrous cap
What are the four most common arteries that are involved with atherosclerosis?
- Abdominal aorta
- Coronary
- Popliteal
- Internal carotid
What are the four major modifiable risk factors for the development of atherosclerosis?
- HTN
- Hypercholesterolemia
- Smoking
- DM
Which gender is more affected with atherosclerosis? Why?
males and postmenopausal women d/t protective effects of estrogen
What is the underlying pathogenesis of atherosclerosis?
macrophages oxidize lipids and are deposited into the intima, leading to proliferation of smooth muscle and sclerotic changes
What are the possible sequelae of stenosis 2/2 atherosclerosis (without rupture)?
- Claudication
- Angina
- Ischemic bowel disease
What percent of an artery has to be occluded before getting symptoms of atherosclerosis?
Greater than 70%
What are the possible sequelae of atherosclerosis (with rupture)?
- MI
- Stroke
What is the histological hallmark of atherosclerotic emboli?
Cholesterol Clefts
What causes aneurysms with atherosclerosis?
Thickening of the vessel wall decreases oxygen penetration, leading to atrophy
What is the pathophysiology of arteriolosclerosis?
Narrowing of small arterioles
What are the two types of arteriolosclerosis?
- hyaline
- hyperplastic types
What causes hyaline arteriolosclerosis? What are the histological characteristics of this?
Protein leaking into the vessel wall, produces vascular thickening
Pink hyaline on microscopy
–What are the two causes of hyaline arteriolosclerosis?–
- Benign HTN forces material into the vessel wall
- DM (nonenzymatic glycosylation of the vessel wall, making it leaky)
What is the major consequence of hyaline arteriolosclerosis in the kidney?
Glomerular scarring, d/t thickening of the afferent arteriole, progressing to CKD
What is the cause of arteriolonephrosclerosis?
Glomerular scarring, d/t thickening of the afferent arteriole
What is hyperplastic arteriolosclerosis? What is the histological appearance of this?
Thickening of vessel wall by hyperplasia of smooth muscle, decreasing the lumen of the vessel
This causes an “onion skin” appearance on microscopy
What causes hyperplastic arteriolosclerosis? What does it result in?
- malignant HTN causes it
- May lead to fibrinoid necrosis of vessel wall, and ARF
What are the classic histological characteristics of hyperplastic arteriolosclerosis in the kidney?
Acute renal failure with a “flea-bitten” appearance
What is monckeberg Medial calcific sclerosis? What is its significance?
Calcification of the media; non-obstructive or clinically significant
What is the classic histological characteristic of Monckeberg medial calcific sclerosis?
dark pink calcification in the intima
Where along the aorta do dissections usually occur? Why here?
Proximal 10 cm since these areas are so thick they need vasa vasora, and these are damaged with HTN
What two common factors in aortic dissections?
- HTN
- preexisting weakness of the media
What is the defect with Marfan’s syndrome?
Fibrillin gene defective, which is the scaffold upon which elastin is laid down
What is the issue with Ehlers Danlos syndrome?
Defective collagen formation
What is the major cause of death with an acute aortic dissection?
Pericardial tamponade
What is the classical infectious disease that causes thoracic aortic dissection? How does it do this?
Tertiary syphilis
Causes end arteritis in the vasa vasorum in the thoracic aorta
Tree bark appearance of the aortic wall = ?
Tertiary syphilis
What is the most common complication a thoracic aortic aneurysm? Why?
Aortic insufficiency d/t pulling on the walls of the aortic root
What is the second most common complication a thoracic aortic aneurysm?
Compression of the mediastinal structures
What is the third most common complication a thoracic aortic aneurysm?
Thrombosis/embolism
Why is it common to get thromboses along the wall of an aneurysm?
Stasis/backflow of blood in the area
What is the most common site for a AAA?
Below the renal arteries, but above the aortic bifurcation
What is the most common way that the abdominal aortic wall becomes weak? How?
Atherosclerosis causes decreased oxygen supply to the wall, causing weakening of the wall
What is the classic patient with AAA?
60+ yo male smokers with a h/o HTN
Over how many cm is an abdominal aortic aneurysm concerning?
When they’re greater than 5 cm in diameter
What is the triad seen with a ruptured AAA?
- *-Hypotension
- Pulsatile abdominal mass
- Flank pain**
What is a hemangioma? What age is it usually seen, and what is the prognosis?
Benign tumor comprised of blood vessels that usually presents at birth and regresses throughout childhood
What are the most common sites of hemangiomas?
Skin and liver
How do you differentiate a hemangioma and purpura?
If blanches with pressure, then it is not a bleed into the skin (it’s a hemangioma)
What is an angiosarcoma? Prognosis?
Malignant proliferation of endothelial cells
Highly aggresive
What are the common sites of angiosarcomas?
Skin
Breast
Liver
What three chemicals have an association with the development of liver angiosarcomas?
- PVC
- Ar
- Thorotrast
What is Kaposi’s sarcoma? What is the cause?
Low-grade malignant proliferation of endothelial cells caused by HHV-8
What is the typical presentation of Kaposi’s sarcoma?
Purple patches, plaques or nodules on the skin, that may involved visceral organs
If you press on a purple patch in Kaposi’s sarcoma, would they blanch? Why or why not?
No, because the blood is in the skin, not the blood vessel itself
What are the three classic patients that get Kaposi’s sarcoma?
- Older eastern european males
- AIDs pts
- Transplant recipients
Pulseless UE in a young asian female = ?
Takayasu’s arteritis
What is the treatment for AIDS patients with Kaposi’s sarcoma?
HAART
What is the treatment for older eastern european males with kaposi’s sarcoma on the skin?
Resect it
What is the treatment for transplant patients with kaposi’s sarcoma on the skin?
Reduce immunosuppression
