Vascular Pathology: Fibromuscular Dysplasia
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Vascular Pathology: Fibromuscular Dysplasia

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Questions and Answers

What is the main characteristic appearance of fibromuscular dysplasia on angiography?

  • Corkscrew pattern
  • Fishnet pattern
  • String of beads (correct)
  • Solid masses

    • What causes luminal stenosis in fibromuscular dysplasia?

  • Increased blood flow
  • Vessel dilation
  • Decreased viscosity
  • Medial and intimal hyperplasia and fibrosis (correct)

    • What type of vessels are primarily affected by fibromuscular dysplasia?

  • Capillaries
  • Elastic arteries
  • Small veins
  • Medium and large muscular arteries (correct)

    • Which factors do endothelial cells produce that influence the vasoreactivity of smooth muscle cells?

    <p>Both vasodilating and vasoconstrictive factors</p> Signup and view all the answers

    What condition can result from renal artery involvement in fibromuscular dysplasia?

    <p>Renovascular hypertension</p> Signup and view all the answers

    What is the known cause of fibromuscular dysplasia?

    <p>Unknown</p> Signup and view all the answers

    What characterizes endothelial dysfunction?

    <p>Alteration in endothelial phenotype</p> Signup and view all the answers

    What nature do certain forms of endothelial dysfunction often take?

    <p>Proinflammatory and prothrombogenic</p> Signup and view all the answers

    What vascular condition can be a complication due to a 'String of beads' morphology?

    <p>Aneurysms</p> Signup and view all the answers

    What is a characteristic feature of a fatty streak?

    <p>It is a collection of foamy macrophages.</p> Signup and view all the answers

    Which vessels are typically spared in atherosclerotic plaque formation?

    <p>Mesenteric and renal arteries (except at their ostia)</p> Signup and view all the answers

    What is the gross appearance of atherosclerotic plaques?

    <p>White-yellow and encroaching on the lumen</p> Signup and view all the answers

    What factor affects the stability or fragility of an atherosclerotic plaque?

    <p>The thickness and ECM content of the fibrous cap</p> Signup and view all the answers

    What can occur as a clinically important change in an atherosclerotic plaque?

    <p>Erosion of the intimal surface leading to thrombosis</p> Signup and view all the answers

    What causes hemorrhage into a plaque?

    <p>Rupture of the thin-walled vessels or overlying fibrous cap</p> Signup and view all the answers

    What happens when blood pressure is too low?

    <p>Tissue death due to inadequate perfusion</p> Signup and view all the answers

    What is a major risk factor for atherosclerosis?

    <p>High blood pressure (hypertension)</p> Signup and view all the answers

    Which of the following blood pressure measurements indicates clinically significant hypertension?

    <p>Diastolic pressure above 80 mmHg</p> Signup and view all the answers

    What does the recruitment of smooth muscle cells (SMC) in response to vascular injury cause?

    <p>Intimal thickening that compromises vascular flow</p> Signup and view all the answers

    What role do endothelial cells play in response to vascular injury?

    <p>They migrate from adjacent uninjured areas to repair damage</p> Signup and view all the answers

    Why is both systolic and diastolic blood pressure important?

    <p>Both are crucial in determining cardiovascular risk</p> Signup and view all the answers

    What is a consequence of high blood pressure?

    <p>End-organ damage</p> Signup and view all the answers

    Which is true regarding blood pressure thresholds?

    <p>There are no rigidly defined threshold blood pressure levels</p> Signup and view all the answers

    What is the consequence of chronic endothelial cell dysfunction?

    <p>Thickening of the intima leading to restricted flow</p> Signup and view all the answers

    What condition does chronic high blood pressure contribute to in the long term?

    <p>Cardiovascular disease</p> Signup and view all the answers

    What is a characteristic feature of atheromas?

    <p>They consist of a raised lesion with a soft grumous core.</p> Signup and view all the answers

    Which of the following factors can lower plasma cholesterol levels?

    <p>Low cholesterol diet</p> Signup and view all the answers

    What is the impact of smoking one pack of cigarettes or more daily?

    <p>It doubles the death rate from ischemic heart disease (IHD).</p> Signup and view all the answers

    How does obesity affect health in relation to HDL levels?

    <p>Decreases HDL levels.</p> Signup and view all the answers

    What class of drugs inhibits HMG-CoA reductase and lowers circulating cholesterol levels?

    <p>Statins</p> Signup and view all the answers

    Which dietary component is known to be beneficial in increasing HDL levels?

    <p>Omega-3 fatty acids</p> Signup and view all the answers

    What is the primary risk factor for left ventricular hypertrophy?

    <p>Hypertension</p> Signup and view all the answers

    Which lifestyle change can reduce the risk associated with cigarette smoking?

    <p>Smoking cessation</p> Signup and view all the answers

    What can moderate ethanol consumption do to HDL levels?

    <p>Increase HDL levels</p> Signup and view all the answers

    What dietary pattern is linked to a higher risk for atherosclerosis?

    <p>Diet high in animal fats and butter</p> Signup and view all the answers

    What triggers chronic inflammation in atherosclerosis?

    <p>Accumulation of cholesterol crystals and free fatty acids</p> Signup and view all the answers

    What is the role of Interleukin 1 (IL1) in inflammation?

    <p>It recruits and activates mononuclear cells</p> Signup and view all the answers

    Which cells are primarily activated in response to chronic inflammation?

    <p>T lymphocytes and macrophages</p> Signup and view all the answers

    What do activated macrophages produce to enhance LDL oxidation?

    <p>Reactive oxygen species</p> Signup and view all the answers

    Which process is driven by growth factors produced by activated macrophages?

    <p>Vascular smooth muscle cell (SMC) proliferation</p> Signup and view all the answers

    What is a consequence of IL1 production during chronic inflammation?

    <p>Local production of cytokines and chemokines</p> Signup and view all the answers

    What initiates the activation of inflammasomes during atherosclerosis?

    <p>Cholesterol crystals and free fatty acids</p> Signup and view all the answers

    What type of immune cells are primarily affected by the cytokines produced during chronic inflammation?

    <p>Mononuclear cells</p> Signup and view all the answers

    What effect do reactive oxygen species from activated macrophages have on LDL?

    <p>They enhance LDL oxidation</p> Signup and view all the answers

    What is primarily recruited to the site of inflammation due to the activation of IL1?

    <p>Mononuclear cells</p> Signup and view all the answers

    Study Notes

    Fibromuscular Dysplasia

    • Focal irregular thickening in medium and large muscular arteries.
    • Affects renal, carotid, splanchnic, and vertebral vessels.
    • Cause unknown.
    • Characterized by medial and intimal hyperplasia and fibrosis, resulting in luminal stenosis.
    • Leads to renovacular hypertension when affecting renal arteries.
    • "String of beads" appearance on angiography.
    • Can lead to vascular outpouching (aneurysms) that can rupture.

    Endothelial Cells (ECs)

    • Influence vasoreactivity of underlying smooth muscle cells (SMCs).
    • Produce both vasodilating (relaxing) and vasoconstrictive factors.
    • Vasodilating factors include nitric oxide (NO).
    • Vasoconstrictive factors include endothelin.

    Endothelial Dysfunction

    • Occurs due to altered endothelial phenotype, seen in various conditions.
    • Often proinflammatory and prothrombogenic.
    • Some forms of endothelial dysfunction are rapid in onset.

    Systemic and Local Blood Pressure

    • Must be maintained within a narrow range.
    • Low pressure leads to inadequate organ perfusion and tissue death.
    • High pressure (hypertension) leads to end-organ damage.
    • Hypertension is a major risk factor for atherosclerosis.

    Clinically Significant Hypertension

    • Individuals with diastolic pressure greater than 80 mmHg or systolic pressures greater than 120 mmHg.

    Atherosclerosis

    • Underlies coronary, cerebral, and peripheral vascular disease.
    • Risk factors include acquired, inherited, and gender- and age-associated factors.

    Atheromas

    • Also known as atheromatous or atherosclerotic plaques.
    • Intimal lesions that protrude into vessel lumens.
    • Raised lesions with a soft, grumous core of lipid covered by a fibrous cap.

    Intimal Thickening

    • Vascular injury, associated with EC dysfunction, stimulates SMC recruitment and proliferation.
    • Leads to intimal thickening that can compromise vascular flow.
    • Endothelial cells migrate from adjacent uninjured areas into denuded areas.

    Chronic Inflammation & Atherosclerotic Lesions

    • Contributes to both initiation and progression of atherosclerotic lesions.
    • Triggered by accumulation of cholesterol crystals and free fatty acids in macrophages and other cells.
    • Leads to inflammasome activation, resulting in IL1 production.

    Interleukin 1 (IL1)

    • Proinflammatory cytokine.
    • Recruits and activates mononuclear cells, including macrophages and T lymphocytes.
    • Leads to local production of cytokines and chemokines.

    Macrophage Activation

    • Activated macrophages produce reactive oxygen species, enhancing LDL oxidation.
    • Elaborate growth factors that drive SMC proliferation.

    T Lymphocyte Activation

    • Participate in the growing atherosclerotic plaque.

    Fatty Streak

    • Collection of foamy macrophages in the intima.

    Atherosclerotic Plaque

    • Intimal thickening + lipid accumulation = plaque.

    Atherosclerotic Plaque: Gross Appearance

    • Color can be white-yellow and encroach on the lumen of the artery.
    • Can be red-brown due to superimposed thrombus over ulcerated plaque.
    • Varies in size and can coalesce to form larger masses.

    Clinically Important Changes in Atherosclerotic Plaque

    • Vessels of the circle of Willis usually spared.
    • Vessels of the upper extremities usually spared.
    • Mesenteric and renal arteries usually spared (except at their ostia).

    Stability of Atherosclerotic Plaque

    • Thickness and ECM content of fibrous cap impact stability and fragility.
    • Influences the tendency to undergo secondary changes.

    Secondary Changes in Atherosclerotic Plaque

    • Rupture, ulceration, or erosion of the intimal surface can induce thrombosis.
    • Thrombus can organize and become incorporated into growing plaque.
    • Hemorrhage into a plaque can rupture the overlying fibrous cap or thin-walled vessels in areas of neovascularization.

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    Description

    This quiz explores the characteristics and implications of fibromuscular dysplasia affecting medium and large muscular arteries. Key concepts include its causes, the 'string of beads' appearance on angiography, and the consequences such as renovascular hypertension. Test your understanding of endothelial cells and their role in vascular health as well.

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