Chapter 11: Vasculature Flashcards
What is the prognosis of gloms tumor aka glomangioma or paraganglioma
Benign
How are AAA typical found
Pulsating mass in the abdomen
What are the morphological features of the Lines of Zahn indicative of
Typically seen on AAA and are indicative of layers of thrombi
Which disease’s pathogenesis is linked to atherosclerosis
-Coronary, cerebral and peripheral vascular diseases
What are the immune cells characteristic of Behcet disease
Neutrophilic
What is the complication commonly seen as a result of churn-Strauss syndrome
Cardiomyopathy, accounting for 50% of the deaths seen with this syndrome
What are the clinical presentations in those with IVC syndrome
LE edema
Distention of the superficial collateral veins of the lower abdomen
What is generally occurring in Hyaline arteriolosclerosis
1) Hypertension causing damage of the epithelium in small blood vessels
2) Plasma proteins leaking across the damaged endothelium
3) Increased smooth muscle matrix synthesis resulting in thickening of the wall and narrowing of the lumen
What is the common location of a glomus tumor
Distal finger
What are the common sites of an angiosarcoma
- Skin
- Soft tissue
- Breast
- Liver
Which patients are AAA commonly seen
60 year old males who smoked
What is a true aneurysm
Intact, but thin, muscular wall at the site of dilation
Saccular- on one side
Fusiform-both sides
Venous thrombosis in the form of thrombophlebitis is commonly seen in which neoplasms
-Mucin producing adenocarcinomas such as lung, pancreas, ovarian
What are the vessels most commonly involved in atherosclerosis in descending order
1) Abdominal aorta
2) Coronary arteries
3) Popliteal arteries
4) Internal carotid arteries
5) Circle of Willis
Which vasculitis is characterized by:
- Medium and small vessels affected
- Lymphocytes, macros, neutrophils
- Eiosinophils*
- Positive ANCA
- History of atopy and asthma
Chung-Strauss syndome
Which vasculitis is characterized by:
- Medium and small arteries affected
- Granulomas*
- Lymphocytes, macros, and neutrophils
Granulamatosis with polyangiitis
Which vasculitis condition is characterized by:
- Necrotizing granulomas of the upper and lower respiratory tracts
- Necrotizing or granulomatous vasculitis of the respiratory tract
- Focal necrotizing, crescentic glomerulonephritis
Granulomatous is with polyangiitis aka Wegener Granulomatosis
Which factors are involved in the inflammation process smooth muscle proliferation and matrix deposition in atherosclerosis
- PDGF
- FGF
- TGF-alpha
Which patients are aortic dissections commonly seen
Hypertensive males between 40-60
What is the outcome in patients with untreated hypertension
50% die of ischemic heart disease or CHF
30% die of stroke
What are the complications seen with Behcet disease
- Neurological involvement
- Rupture of aneurysms
What is the prognosis of lymphagiomas
Benign
What is the pathogenesis of superior vena cava syndrome
Neoplasms compress or invade the superior vena cava, resulting in the dilation of veins in the neck, head, and arms, along with cyanosis
What is a false aneurysm
Defect through the wall fo the vessel that communicated with an extravascular hematoma
What the mechanism of pathogenesis in Marfans
1) Results in the decreased synthesis of fibrillin
2) Decreased fibrillin results in increased TGF-Beta activity
3) Decreased activity results in weakened walls
Which vasculitis is characterized by:
- Small arteries, capillaries, and veins
- May or may not contain immune cels
Leukocytociastic vasculitis
What are the common complications seen as a result of Kawasaki disease
Aneurysms, leading to thrombosis/rupture, causing an acute MI
Which cells are primarily recruited in the inflammation of atherosclerosis
Macrophages and T cells
Which conditions can be caused by tertiary syphilis
- Thoracic aorta aneurysms
- Aortic valve annulus aka regurgitation
Granulomatosis with polyangiits lesions are characterized by what
- Geographic patterns of central necrosis, usually in the upper respiratory tract
- Cavitation lesions and necrotizing granulomas in the lower tract
What is the main risk factor for aortic dissection
Hypertension
What is occurring during fibromuscular dysplasia
Focal irregular thickening of the medium and large muscular arteries
What is the complication seen with superior vena cava syndrome with regards to pulmonary vessels
Respiratory distress
Which autoantibody is commonly seen in microscopic polyangitis
MPO-ANCA aka p-ANCA
Which vasculitis is characterized by a patient older than 40 with polymyalgia rheumatica
Giant cell arteritis
What is the prognosis of an angiosarcoma
Malignant endothelial tumor
What is the stereotypical response of a vessel wall to any injury/insult
Intimate thickening due to activation of smooth muscle cells
What conditions can cause secondary raynaud phenomena
SLE
Scleroderma
Thromboangiitis obliterans
*All make it worsen with time
Which patient population is seen to have takayasu arteries
Younger (<50)
Which vessels are commonly used for vascular intervention
Saphenous vein (50% patancy after 10 years) or left internal mammary artery (90% patency)
What are the factors that determine cardiac output
Heart rate and the stroke volume (determined by blood volume aka sodium excretion)
What are the characteristics of Kaposi sarcoma caused by: Endemic African from
Patients <40, involving the LN
What are the presenting features of Kawasaki disease
- young child (<4)
- Erythema of the conjunctiva, oral mucosa, palms and feet
- Cervical lymph node enlargement
Hypertension causes ischemia in which portion of the vessel
Outer media
What is the common location for an aortic dissection
Ascending aorta, within 10cm of the aortic valve
What is vascular resistance influenced by
- At the levels of arterioles
- By neural and hormonal inputs
Why is it important to distinguish between infectious and non-infections vasculitis
- Non-infectious vasculitis is treated with immune suppression
- Infectious is treatment of the underling cause
What is the size cut off for AAA that are managed surgically
5 cm
Which neoplasms tend to cause IVC syndome
Hepatocellular carcinomas (HCC) Renal cell carcinoma (RCC)
What is the treatment for Behçet’s disease
Immunosuppression with steroid or TNF antagonist
What are the characteristics of Kaposi sarcoma caused by: classic form
Older men from Middle eastern, Mediterranean or Eastern European descent
Which vessels are involved in Kawasaki disease
Coronary arteries
What is the histological finding in hyperplastic arteriolosclerosis
Concentric lamellations that give an onion skin appearance
What is the prognosis of a patient with Kawasaki disease
Self limiting, but given IVIg or aspirin for the lowered coronary risk
What is the heritability of Marfans
Autosomal dominant
Which autoantibody is commonly seen in polyangiitis
PR3-ANCA aka c-ANCA
What are the steps and components in process of fatty streaks and atherosclerotic plaque formation
1) Endothelial injury due to something, usually the hyperlipidemia
2) Epithelial dysfunction results in an inflammation process and immune cell recruitment
3) lipids accumulate in the intima and are taken up by macrophages, resulting in oxidation of lipids and damage
4) LDL accumulates within macrophages and smooth muscle cells forming foam cells and fatty streak
5) Smooth muscle cells proliferate and provide the fibrous cap, which forms the full atherosclerotic plaque
What is generally occurring in hyperplastic arteriolosclerosis
Severe hypertension causes the smooth muscle cells to proliferate and form concentric lamellations that result in narrowing of the lumen
What are the locations that atherosclerotic plaques tend to accumulate
- Openings of the exiting vessels
- Branch points
- Posterior abdominal aorta
What is the initial event following vascular injury
Activation of the endothelial cells
What is the pathogenesis of varicose veins
-Abnormal dilation of veins with valvular incompetence or secondary to sustained intraluminal pressure
Which vasculitis condition is characterized by:
- Aphthous ulcers of the oral cavity
- Genital ulcers
- Uveitis
Behcets
What is the major cause of noninfectious vasculitis
Immune response, either local or systemic
What is the pathogenesis and cause of bacillary angiomatosis
Vascular proliferation in a response to the gram negative Bartonella bacilli
What is the mechanism of pathogenesis in fibromuscular dysplasia
Medial and intima hyperplasia leading to luminal stenosis
What is cystic medial degeneration and what is causing it
- Loss of vascular wall elastic tissue by damage or decreased synthesis
- increased proteoglycan deposition
*Leads to the increased risk of aneurysms
Which patient history is seen in Buerger disease, and thromboangiitis obliterans
Heavy smokers <35
In the cause of vasculitis in a patient with hepatitis B, which condition should be considered
Polyarteritis nodosa, as commonlt seen in HBsAg
What is the most common vascular hemangioma
Capillary
What is the step by step process of response to vascular injury
1) Activation of the endothelium
2) Recruitment of the smooth muscles cells to the intima
3) Smooth muscle cells under mitosis and proliferation
4) The extracellular matrix is elaborated
What are the baseline blood pressure reading that are considered hypertensive and lead to increased with of atherosclerosis
139/89
What is the finding on the appearance of the kidney in the case of essential hypertension
- Granular appearance due to the chromic increased BP
- Renal afferent arteriole is hyalinzed
What is the relation between the ANCA titer and severity of disease
Generally correlate
What condition are large cavernous lymphangiomas of the neck commonly seen in
Turner syndrome
Which patients are commonly seen to have bacillary angiomatosis
Immunocompromised
What are the clinical presentations in someone with Raynaud phenomenon
-Young women, usually as a result of cold, or stress
Which patient gorup is commonly affected by Giant cell arterietis
Older population
Which conditions commonly are seen with immune complex vasculitis
- SLE
- Drug hypersensitivity
- Secondary exposure to infectious agent (polyarteritis nodosa with HepB)
What are the complications seen with Takayasu arteritis
Aortic arch and major branch vessel involvement leading to dilation and valve insufficiency
(Ex. Narrowing of the brachiocephalic, carotid, and subclavian arteries)
Tertiary syphilis causes ischemia to which portions of the vessel
Outer media
What is the common cause leading to angiosarcomas
Radiation
Hepatic form is due to toxins such as pesticides, thorotrast, polyvinyl chloride
What is the angiographic findings in a patients with fibromusclar dysplasia
String of beads
Which antibody is commonly associated with microscopic polyangiitis
MPO-ANCA
Which conditions are commonly seen with churg-Strauss syndrome
Asthma, allergic rhinitis, hypereosinophilia
The endothelial marker CD31 is indicative of which vascular tumor
Angiosarcoma
What is the treatment of a patient with microscopic polyangiitis
Immunosuppression increased long term survival
What is the pathogenesis of immune complex vasculitis
Autoantibody production and formation of immune complexes that deposit in the vascular wall
Thoracic aortic aneurysms are commonly caused by which conditions
Hypertension, but can be from Marfans
Marfan is caused by a defect in which component
Fibrillin synthesis
What is a common condition seen in as a result of fibromuscular dysplasia
- Renovasular hypertension due to stenosis of the renal arteries
- Anearysms that may rupture
What is the correlation of blood pressure with age
Are age increases, the blood pressure increases due to loss of elastic components of the arteries
What is the treatment for granulomatosis with polyangiitis
Steroids, cyclophosphamide, TNF antagonists
What is the prognosis of hemangiomas
Benign vascular tumors
Which vasculitis condition is characterized by:
- T cell mediated damage
- Inflammation of temporal arteries
Giant cell arteriitis and aortitis
Which cytokine is released in the inflammation of atherosclerosis
IL-1
What are the lesions seen in bacillary angiomatosis
Localized red papules due to the proliferation of capillaries with plump endothelial cells
Which vasculitis condition is characterized by:
- Ocular disturbances
- Weakening pulses and BP of the upper extremities
- Granulomatous of the medium and large arteries
Takayasu arteritis
Which type of necrosis is involved in microscopic polyangiitis
Segmental Fibrinoid necrosis
What is the finding on the appearance of the kidney during malignant hypertension
- Nutmeg appearance with tiny Hemorrages
- Onion skinning appearance in the renal arteriole
What are the characteristics of the appearance of lesions in Churg-Strauss
Necrotizing as seen in PAN and microscopic polyangiitis, but with the addition of eosinophils and granulomas
Which vessels are commonly involved in Microscopic polyangiits
Necrotizing vasculitis of the arterioles, capillaries and venules*
What is the histological finding in Hyaline arteriolosclerosis
Homogenous pink (hyaline) thickening of the vessel walls
Which specific vessels in particular are affected in fibromuscular dysplasia
Renal, carotid, Splanchnic and vertebral vessels
What are the characteristics of Kaposi sarcoma caused by: Transplant form
Assocaited with T cell suppression following transplantation
What condition is characteristics of the features:
- 40 year old male
- Persistent pneumonitits and sinusitis
- Renal disease
- Nasopharyngeal ulcerations
Granulomatosis with polyangiitis
What is the stain and test run to identity the cause of bacillary angiomatosis
-Warthin-starry stain or PCR
What are the characteristics of an atherosclerotic plaque
- Necrotic center with cell debris, cholesterol crystals, calcium and foam cells
- Fibrous cap (contains smooth muscle cells, macrophages, ECM components, foam cells), overlying the necrotic center
What is the complication seen when there is renal vein involvement in IVC syndrome
Massive proteinuria
What are the characteristics of the cell types involved in Giant cell arteritis
Lymphocytes and macrophages
What are the conditions that commonly cause secondary hypertension
- Primary hyperaldosteronism
- Cushing
- pheochromacytoma
- renal artery stenosis leading the increased Renin
What is the pathogenesis of inferior vena cava syndrome
-Neoplasms compress or invade the IVC or thrombosis of the hepatic renal or LE
In the case of secondary hypertension due to renal artery stenosis, what can be found
- Increased renin production
- Bruit on the side of the renal stenosis
What are the clinical results of varicose veins
Stasis, congestion, thrombus, emboli and ischemia overlying skin resulting in stasis dermatitis
*Very rare for the superficial veins to cause an embolism like one from a deep vein
What are the vessels involved in polyarteritis nodosa
Small and medium sized vessels
Which patient group is seen to have Kawasaki disease
Young children (<4)
If the diameter of an arteriole increases by two, was is the corresponding change in blood pressure
Increases by 16 due to the change in resistance to the fourth power
What are the characteristics of a epithelioid hemangioendothelioma
What is the mechanism that syphilis can cause aneurysms
1) Late state syphilis leads to obliterative endarteritis in small vessels, including the vasa vasorum of the thoracic aorta
2) This causes ischemia of the aorta, leading to weakening of the walls and formation of an aneurysm
What is the histological finding in Kaposi sarcoma
Sheep’s of spindle cells
What is the histological finding with microscopic polyangiitis
Apoptotic neutrophils aka fragmented PMN
What are the characteristics of the lesions seen in polyarteritis nodosa
Segmental transmural necrotizing inflammation (neutrophils, eosinophils, lymphocytes, and macros) at the branch points
*Lesions typically not circumferential
What is the prognosis of epithelioid hemangioendothelioma
Intermediate grade vascular tumor
What is the pathogenesis of raynaud phenomenon
Vasospasms of the small arteries and arterioles in the fingers and toe, especially in the cold
What is the treatment for bacillary angiomatosis
Macrolides
How can primary and secondary raynaud phenomenon be differentiated
Primary- symmetrical involvement
Secondary- asymmetrical involvement
Which characteristic is required fro churn-Strauss syndrome
Eosinophils
What are the characteristics of Kaposi sarcoma caused by: AIDs associated
-Most common form in the US, usually spread through lymph nodes
What is the immune cell type seen in granulomatosis with polyangiitis
T cell mediated hypersensitivity
What is unique about the pauci immune ANCA mediate process
Does not form circulating immune complexes, and the vasculitis lesions do not contain the antibody and complement
Which vasculitis condition is characterized by:
- Multiunucleated giant cells
- Fragmentation of the elastic lamina and thickening
- Patchy and focal inflammation of vessels
Giant cell arteritis
What are the two conditions of small vessels that can be caused by hypertension
1) Hyaline arteriolosclerosis
2) Hyperplastic arteriolosclerosis
What are the places that mycotic aneurysms can originate from
- Septic emboli (usually from infective endocarditis)
- Circulating organisms infecting the arterial wall
What is the antibody seen with granulomatosis with polyangiitis
PR3-ANCA (95%)
What is the major risk factors of atherosclerosis
Hypercholesteremia
*Is significant to cause lesion development without any other factors
What are the common causes of cystic medial degeneration leading to aneurysms, and what are the two most important
- Atherosclerosis
- Hypertension
- Elastin decreased diseases (Marfan)
- Mycotic
What is the cause of small bore artery replacement failure
Thrombosis or intima hyperplasia
What is the common location of AAA
Below the renal arteries, usually involving the iliac arteries
What is the typical cause of abdominal aortic aneurysm
Atherosclerosis
In a patient with arteriovenous malformations, which outcome is of most concern
High output cardiac failure due to the shunting of blood from the arterial side to the venous side, resulting in the increased workload of the heart
What are the outcomes of the activated endothelial cells following injury
- Procoagulants
- Adhesion molecules
- Growth factors
- Vasoactive factors
What is the overall concern with atherosclerosis and at which point does it get reach
Stenosis leading to ischemia, which is known as critical stenosis, aka 70% occlusion
Which vasculitis condition is characterized by:
-Segmental, thrombosing vasculitis of small and medium vessels, especially the tibial and radial arteries
Buerger disease
What is the significance of multiple risk factors in the cause of atherosclerosis
They are synergistic rather than just additive
During thromboangiitis obliterans aka Buerger disease, what is the common histological finding
Thrombus with small microabscesses composed of neutrophils surrounded by granulomatous inflammation
What are the factors in metabolic syndrome that go to increase the risk of atherosclerosis
- Insulin resistance
- Hypertension
- Hypercoagulobility
- dyslipidemia
What is occurring in Monckeberg medial sclerosis
Calcification of internal elastic membrane in muscular arteries of patients older than 50
*There is no narrowing of the lumen or clinical significance
What type of necrosis is commonly seen in polyarteritis nodosa
Fibrinoid necrosis
Which vasculitis is characterized by:
- Small and medium sized arteries
- Capillaries
- Veins
- Neutrophils*
- May contain lymph, macros, or eosinophils
- Congenital ulcers*
Behcet disease
What are the characteristics of glomus tumor
Smooth muscle in origin, usually in the distal fingers
-Benign and painful
Which patient population is at higher risk for essential Hypertension
Older patients
African Americans
Which autoantibody is commonly seen in Churg-Strauss
MPO-ANCA aka p-ANCA
Which lipids tend to be present in atherosclerotic plaques and what is their location
Cholesterol and cholesterol esters located in the intima
IN the case of vasculitis due to drug hypersensitivity, what is the next step
Stop administering the drug, as it will resolve the vasculitis
Polyarteritis nodosa is commonly seen in assocaited with which condition
Hepatitis B
Which condition or form of vasculitis is present if the aorta is involved
Giant cell arthritis
Which patient population is more readily affected by fibromuscular dysplasia
Young women, with first degree relatives increasing chances
What locations of vessels are commonly seen to be affected by microscopic polyangiitis
Renal glomeruli and lung capillaries
What is the cause of Karposi sarcoma
HHV8
Atherosclerosis causes ischemia of which portion of the vessel
Innermedia
Which halotype is associated with Behcet disease
HLA-B51
What is the prognosis of a patient with untreated granulomatosis with polyangiitis
80% mortality at 1 year
What is the secondary complication seen with Giant cell arteritis
Double vision or damage to the ophthalmic artery leading to vision loss
Which vasculitis is characterized by:
- Medium and small sized arteries
- Veins
- Thrombosis*
- Usually a young male smoke
Buerger disease
Which vessels are commonly affected in polyarteritis nodosa (PAN)
Renal, heart, liver, and GI
Pulmonary vessels are spared
What is the classical presentation of aperitif dissection
-Sudden onset of chest pain that readiates to the back between the scapulae, while moving downward as the dissection progresses