Vascular Pathology Flashcards

1
Q

Large-vessel vasculitis

A

involves the Aorta and its major branches

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2
Q

Medium-vessel vasculitis

A

Involves muscular arteries that supply organs

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3
Q

Small-vessel vasculitis

A

Involves arterioles, capillaries, and venules

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4
Q
  • Involvement of **branches of carotid artery, large to small arteries **of the head, intimal thickening, Granulomatous
  • Older adults ( > 50 y.o.), mostly females
  • Headache (temporal artery involvement)
  • Visual disturbances (opthalmic artery involvement)
  • Jaw claudication
  • Flu-like symptoms with polymyalgia rheumatic
  • ESR elevated and T-cell mediated immune response
  • Biopsy: giant cells and intimal fibrosis, segmental lesions
  • Tx: corticosteroids, high risk for blindness, surgery
A

Temporal arteritis

(Giant Cell)

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5
Q
  • Granulomatous vasculitis (Aortic arch branch points) and Coronary and Renal arteries, aneurysm formation and dissection: Large to Medium
  • Transmural fibrous thickening of Aorta and branches
  • Adults ( < 50 y.o.), young asian females, and children
  • Visual and Neurologic symptoms
  • pulseless disease’ - weak or absent **upper extremity, **fever night sweats, arthritis, myalgias, skin lesions
  • ESR is elevated
  • Tx: corticosteroids
A

Takayasu Arteritis

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6
Q
  • Fibrinoid necrotizing vasculitis, multiple organs w/ varying stages of acute, chronic, and fibrosing lesions in Medium to Small arteries among All Ages
  • Lungs are NOT involved, NOT a/w ANCA
  • Young adults (w/ HTN) (renal artery involvement)
  • Mesenteric artery involvement (abdominal pain w/ melena (black ‘tarry’ feces)
  • Neurologic disturbances and headache
  • Skin lesions, weight loss, malaise, microaneurysms
  • A/w Hepatitis B Surface Antigen (HBsAg), HBV
  • Transmural inflammation - String of pearls’
  • Tx: corticosteroids and cyclophoshamide, FATAL
A

Polyarteritis Nodosa

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7
Q
  • Asian, children < 4 years old
  • Large to Small arteries - ‘strawberry tongue
  • Non-specific signs: Acute febrile illness ‘Fever’, Conjuctivitis, Erythematous ‘skin’ rash on palms and soles, cervical lymph nodes , Lymphadenopathy
  • Coronary artery involvment
    • Thrombosis w/ myocardial infarction
    • Aneurysm with rupture
  • Tx: Aspirin (TXA2) and IV immunoglobulin- disease is self-limited
A

Kawasaki Disease

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8
Q
  • Necrotizing vasculitis involving the digits
  • Ulceration, gangrene, and autoamputation of fingers and toes (Small / Medium sized arteries of extremities)
  • Superficial nodular phlebitis
  • Thrombus and microabscesses
  • Raynaud phenomenon is often present
  • Males, < 40 y.o.
  • Associated with heavy smoking
  • Tx: smoking cessation
A

Buerger Disease

(Thromboangioitis obliterans)

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9
Q
  • Non- and Necrotizing granulomatous vasculatiis:
    • Nasopharynx, Lungs, and Kidneys (‘palisading’)
  • Middle aged male w/ sinusitis or nasopharyngeal ulceration (mainly adults), otitis media, mastoiditis
  • Hemoptysis with bilateral nodular lung infiltrates
  • Hematuria due to ‘rapidly progressive glomerulonephritis’
  • Serum c-ANCA / PR3-ANCA correlate w/ disease activity
  • Biopsy: large necrotizing granulomas w/ adjacent necrotizing vasculitis of small arteries, veins, capillaries
  • Tx: cyclophosphamide and steroids, common relapse
A

Wegener Granulomatosis

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10
Q
  • Necrotizing vasculitis involving multiple organs
    • arterioles, capillaries, and venules w/ **leukocytoclastic vasculitis **(Fragmentend / apoptotic neutrophils)
    • Lung and Kidney, hemoturia, hemoptysis
  • **MPO-ANCA / **p-ANCA **correlate w/ disease activity
  • Nasopharyngeal involvement and granulomas absent
  • Tx: corticosteroids and cyclophosphamide, immunosuppresion, Relapse is common
A

Microscopic Polyangiitis

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11
Q
  • Necrotizing granulomatous inflammation w/ Eosinophils involving multiple organs (esp. Lungs, Heart, Kidney and GI (pauciimmune glomerulonephritis))
  • **Asthma, sinusitis, palpable purpura, **and peripheral eosinophilia / neuropathy (wrist / foot drop)
  • Serum MPO-ANCA / p-ANCA correlate with disease activity, increased IgE levels
A

Churg-Strauss Syndrome

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12
Q
  • ​Vasculitis due to IgA immune complex deposition
  • Most common vasculitis in children (2 - 6 y.o.)
      1. Palpable ‘purplish/red’ purpura on Buttocks (‘trunk’) and Legs (‘extremities’)
    1. GI pain, melena, multiple lesions, and bleeding
    2. Arthralgias
  • Hematuria (IgA vasculitis / nephropathy)
  • Following upper respiratory tract infection
  • Self-limited
  • Tx: steroids
A

Henoch-Schönlein Purpura (HSP)

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13
Q
  • Stenosis decreases blood flow to glomerulus
  • ATII raises blood pressure
    1. ​Contracting arteriolar smooth muscle –> increaseing total peripheral resistance
    2. Promoting adrenal release of Aldosterone, increases resorption of Na+ in distal convoluted tubule (expanding plasma volume)
  • HTN with increased plasma Renin and unilateral atrophy (low blood flow) to affected kidney
  • Different for Elderly Men and Young Females
A

Renal Artery Stenosis

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14
Q

Renal Artery Stenosis:

Elderly Men –> __________

Young Women –> __________

A

Renal Artery Stenosis:

Elderly Men –> Atherosclerosis

Young Women –> Fibromuscular dysplasia

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15
Q
  • Blood pressure > 200 / 120 mmHg
  • Acute end-organ damage
    • Acute Renal failure
    • Headache
    • Papilledema
A

Malignant HTN –> medical emergency

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16
Q
  • Med/Lrg arteries w/ Intimal plaque
    • Necrotic Lipid core w/ fibromuscular cap
    • Often undergoes dystrophic calcification
  • Abdominal aorta, Coronary artery, Popliteal artery, Internal carotid artery are commonly affected
  • (4) Modifiable risk factors: HTN, Hypercholesterolemia (LDL increase, HDL decrease), Smoking, Diabetes
  • Nonmodifiable: Age, Gender (Males and Postmenopausal), Genetics (History)
  • Lipids oxidized –> macrophage laden –> Fatty Streaks (arise in teenagers) involves PDGF and FGF prolif.
A

Atherosclerosis

17
Q

Complications of Atherosclerosis

A
  • Peripheral vascular disease (extremity arteries)
  • Angina (coronary arteries)
  • Ischemic bowel disease (mesenteric arteries)
  • Thrombosis –> myocardial infarction (coronary)
  • Thrombosis –> stroke (middle cerebral artery)
  • Plaque rupture w/ embolization –> embolus
  • Weakening can result in aneurysm
18
Q
  • Proteins leaking into the vessel wall producing vascular thickening (pink on microscopy)
  • Consequences of long-standing:
    1. ​Benign HTN (‘Systemic HTN’)
    2. Diabetes
  • Associated with Nephrosclerosis –> Renal Failure
  • Reduced vessel caliber w/ end-organ ischemia producing glomerular scarring (arteriolonephrosclerosis) that slowly progresses to Chronic Renal Failure
A

Hyaline Arteriolosclerosis

19
Q
  • Thickening of the vessel wall by hyperplasia of smooth muscle (‘onion skin’ appearance)
  • Acute w/in 1-2 years
  • Consequence of Malignant HTN
  • Reduced vessel caliber with end-organ ischemia
  • May lead to fibrinoid necrosis of the vessel wall w/ hemmorrhage –> acute renal failure w/ “flea-bitten’ appearance
  • Life threatening organ damage: Brain, Kidneys, Heart –> Medical emergency
A

Hyperplastic arteriolosclerosis

20
Q
  • Calcification of the media of the arteries, especially the Radial or Ulnar (medium sized) arteries
  • Nonobstructive
  • Usually benign; “pipestem” arteries on x-ray
  • Intima not involved
  • Not clinically significant; seen as an incidental finding on x-ray or mammography
A

Mönckeberg medial calcific sclerosis

21
Q
  • Dissection through the media of the aortic wall
  • Proximal 10 cm of the Aorta w/ preexisting weakness
  • HTN (older adults), Bicuspid aortic valve
  • Connective tissue defects (younger individuals)
    • Marfan Syndrome –> defective Fibrillin –> cystic medial necrosis of the Aorta
    • Ehlers-Danlos Syndrome –> defective Collagen
  • Sharp, tearing chest pain that radiates to the back
  • Complications: Pericardial tamponade (death), Rupture w/ fatal hemorrhage, Obstruction of branching arteries (coronary and renal) –> end-organ ischemia
A

Aortic Dissection

22
Q
  • Balloon-like dilation of Thoracic Aorta
  • A/w Cystic medial degeneration due to HTN (elderly)
  • Tertiary syphilis –> Obliterative Endaorteritis of the vasa vasorum results in luminal narrowing, decreased flow, and atrophy of the vessel wall
  • ‘tree-bark’ appearance of the Aorta
  • Complication: Dilation of the Aortic valve root → Aortic valve insufficiency → pain
  • Compression of the mediastinal structures (airway or esophogus) → shortness of breath
  • Thrombosis / embolism
A

Thoracic Aortic Aneurysm

23
Q
  • Balloon-like dilation arises below the Renal arteries but above the Aortic bifurcation
  • Primarily due to Atherosclerosis: Males, Smokers, > 60 years old, HTN
  • Atherosclerosis increases diffusion barrier to the media
  • -> atrophy and weakness of the vessel wall
  • Pulsatile abdominal mass that grows with time
  • Complications: when > 5 cm –> triad of hypotension, pulsatile abdominal mass, and flank pain
  • Compression of structures (ureter), Thrombosis/emboli
A

Abdominal Aortic Aneurysm (AAA)

24
Q
  • Benign tumor comprised of blood vessels, Red papules or nodules
  • Blanches when pressed
  • Commonly present at birth
  • Regresses during childhood
  • Involves the Skin and Liver
A

Hemangioma

25
Q
  • Maliginant proliferation of Mesenchymal endothelial cells
  • Highly aggressive, poorly demarcated, variegated, fleshy masses, atypical cells lining vascular lumen
  • Older Adults (M = F), CD 31 / 34 Postiive
  • Common sites: Skin, Breast, and Liver
    • a/w Rx therapy after mastectomy
  • Polyvinyl chloride (PVC) exposure to Liver
  • Arsenic exposure
  • Thorotrast exposure (radiocontrast agent)
  • Poor prognosis
A

Angiosarcoma

26
Q
  • Low-grade malignant proliferation of endothelial cells
  • Locally aggressive
  • Purple patches, plaques, and nodules on the skin
  • May involve visceral organs
  • HHV-8 virus infects endothelial cells
  • Seen in:
    • Older Eastern European Males –> Surgery
    • AIDS –> antiretroviral agents
    • Transplant recipients –> decreasing immunosuppresion
A

Kaposi Sarcoma

27
Q
  • Directed toward neutrophil azurophilic granules (to increase PMN activity) a/w microbial peptides (polyangiitis)
  • Primarily involves intracellular component –> plasma membrane expression –> leads to vascular injury
A

Anti-proteinase-3 (PR3-ANCA)

Non-infectious Vasculitis

28
Q
  • Directed toward lysosomal granules (oxygen free radical formation)
    • Drug to cross-reactive microbial antigens
    • Infection (elicit cytokines: TNF) & endotoxic exposure
    • Autoantibodies (direct endothelial cell injury or reactive neutrophils
  • Leads to vascular injury
A

Anti-myeloperoxidase
(MPO-ANCA)

Non-infectious Vasculitis

29
Q

Benign Vascular Tumors

A
  • Ectasias
  • Telangiectasias
  • Hemangiomas
  • Pyogenic granulomas
  • Lymphangiomas
  • Glomangiomas
  • Bacillary angiomatosis
30
Q

Intermediate-grade Vascular Tumors

A
  • Kaposi Sarcoma
  • Hemangioendothelioma
31
Q

Malignant Vascular Tumors

A
  • Angiosarcoma
  • Hemangiopericytoma
32
Q
  • Plaques or nodules composed of Lipid-laden Histocytes in the skin
  • Especially the eyelids
  • Looks like a rash of raised red nodules on the skin and fatty orange / yellow plaques on the eylids
A

Xanthomas

33
Q
  • Lipid deposits in Tendon, especially Achilles
A

Tendinous Xanthoma

34
Q
  • Lipid deposit in Cornea
    • Appears early in life w/ Hypercholesterolemia
    • Cholesterol deposits in or hyalinosis of the corneal stroma
  • Common in elderly (Arcus Senilis) and fades in infants
  • A/w decreased blood flow to eye
  • Appears as a white, grey, or blue opaque ring in the corneal margin (peripheral corneal opacity), or white ring in front of the periphery of the iris.
A

Corneal Arcus

35
Q
  • Systolic BP > 140 mmHg and/or Diastoloic > 90 mmHg
  • Age, Obesity, Diabetes, Smoking, Genetics, Black > White > Asian
  • 90% is Essential due to CO and TPR
  • 10% is Secondary; Renal disease, including Fibromuscular dysplasia in young pts.
  • A/w Atherosclerosis, LVH, Stroke, CHF, Renal failure, retinopathy, and Aortic Dissection
A

Hypertension (HTN)

36
Q
  • Common
  • Two types:
    • Hyaline (thickening of small arteries in essential HTN or Diabetes mellitus)
    • Hyperplastic (‘onion skinning’) as seen in severe HTN
A

Arteriolosclerosis