Vascular Pathology Flashcards

1
Q

Glomus

A

Benign vascular tumor arising from specialized smooth muscle cells of the glomus body

  • often found on fingertips, under the nai
  • painful
  • usually biopsied to r/o melanoma
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2
Q

Complications of Atherosclerosis?

A
  • Ischemia
  • MI
  • Stroke
  • Aortic Aneurysms
  • Peripheral vascular disease
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3
Q

Kawasaki Disease

  • symptoms?
  • epidemiology?
  • Results in what disease if untreated?
A
  1. Conjunctivitis
  2. Rash
  3. Adenopathy (cervical)
  4. Strawberry tongue
  5. Hands and Feet changes
  6. Fever
  • Usually affects Asian children
  • Results in Coronary Aneurysm or MI if untreated
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4
Q

Disease characterized by the triad of:

  1. Recurrent Aphthous Ulcers
  2. Genital Ulcers
  3. Uveitis (pigmented eye)
A

Behget’s Disease

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5
Q

Bacillary Angiomatosis

A

Reactive vascular proliferation caused by gram neg bacteria:
1. Bartonella henselae - found in cats (causes Cat-scratch disease in immunocomprimised)

  1. Bartonella quintana - transmitted by human body lice
    - bacteria induce HIF-1alpha
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6
Q

Hemangioma

A

Benign Vascular tumor arising from endothelium

  • composed of blood-filled vessels
  • very common
  • malignant transformation is rare
  • 4 subtypes
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7
Q

Hereditary hemorrhagic Telangiectasia (Osler-Weber-Rendu disease)

A

Benign Vascular Ectasia
- caused by mutation in TGF-beta signaling to endothelial cells

-nosebleed is most common symptom

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8
Q

Sturge-Webber Syndrome

A

Vascular ectasias

  • Port wine colored skin patches in trigeminal nerve distribution
  • may also have ipsilateral venous angiomas in the cortico leptomeningies

may lead to metal retardation, seizures

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9
Q

What are AGEs

A

Advanced glycation end products

  • forms from the non-enzymatic rxn between reducing sugars (i.e. glucose) and proteinsm lipids or nucleic acids
  • implicated in numerous diabetes and age-rlated diseases.
  • first identified = glycated hemoglobin (HgbA1-C)
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10
Q

A Thoracic Aneurysm is most likely caused by?

A

Syphilis

Results in “tree-bark” appearance of aorta

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11
Q
  • Granulomatous, classically involving the heart
  • adults
  • weak/absent pulse in upper extremities
A

Takayasu Arteritis

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12
Q

Causes of an Aortic dissection?

A

Inadequate or abnormal connective tissue synthesis (Proteoglycans, collagen, elastin)

  1. HTN - causes hyaline arteriosclerosis of the vaso vasorum which leads to atrophy/weaking of the media (Most common cause)
  2. mutations in TGF-Beta receptor or downstream signaling
  3. Collagen defect diseases (Marfans and Ehlers-Danlos syndrome)
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13
Q

Angiosarcoma

A

Vascular tumor arising from endothelium

  • aggressive tumors
  • 30% have 5 yr survival
  • occurs in older adults
  • younger PTs w/ smaller lesions have better prognosis
  • Skin, soft tissue, breast, and liver are most common sites
  • Histo: CD31+
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14
Q

An ascending aneurysm is most likely caused by:

A

HTN

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15
Q

ectasia (definition)

A

Local dilation of a structure

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16
Q

Fibromuscular dysplasia

A

Congenital vasculature anomaly characterized by:

  • Alternating thickened and thinned arterial walls -> “string of beads” appearance
  • media of arterial walls
  • affects small to medium sized vessels
  • commonly bilateral
  • non-inflammatory, non-atherosclerotic
  • occurs most commonly in younger women
  • if occurs in renal artery, it can lead to renovascular HTN
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17
Q

Buerger Disease is caused by what?

  • symptoms?
  • Tx?
A

Smoking => inflammation and thrombosis of arteries supplying the hands and feet

Symptoms:

  • claudation (cramping)
  • Cold sensitivity
  • gangrene w/ amputation of digits

Tx: stop smoking

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18
Q

Modifiable risks of atherosclerosis

A
  1. HTN
  2. Hypercholesterolemia
    - high LDL
    - Low LDL
  3. smoking
  4. Diabetes
  5. Chronic inflammatory diseases
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19
Q

Name two Large-vessel Vasculitis diseases?

A
  1. Temporal (Giant cell) Arteritis

2. Takyasu Ateritis

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20
Q

Capillary Hemangioma (characteristics)

A

Most common form of hemangioma

  • Affects Skin, subQ, mucus membranes, liver, spleen, and kidney
  • histo: Thin walled w/ scant stroma
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21
Q

Characteristics of malignant vascular tumors:

A
  1. Usually do not form well organized vessels
  2. More cellular, more cytologic atypia
  3. may need confirmation with CD31, 34, and D2-40
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22
Q

Tx of Granulomatosis w/ Polyangitis (Wegner’s)

A

Cyclophosphamide and Corticosteriods

” 3 C’s of Wegners” - (drugs and C-ANCAs)

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23
Q

Name 3 medium vessel vasculitis diseases

A
  1. Polyarteritis Nodosa
  2. Kawasaki Disease
  3. Buerger Disease
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24
Q

Berry Aneurysms

A

Congenital vascular anomaly characterized by:
- Thin-walled arterial outpouching in cerebral vessels
- classically occurring at branched points (circle of Willis)
- Media is attenuated (thinned)
*can rupture and cause fatal intracerebral hemorrhage
Buzzers: “worst headache of my life”

25
Q

T or F: Aneurysm involves only the media

A

FALSE!

Aneurysm involves ALL 3 layers

26
Q

Constitutional/nonmodifiable risks of atherosclerosis

A
  1. Genetics
  2. Age
  3. Gender (pre-menopausal women protected)
27
Q

What two diseases are associated with Arteriosclerosis?

A
  1. HTN

2. DM

28
Q
  • Granulomatous vasculitis
  • occurs in older white females
  • presents w/ headache, visual disturbances, and jaw claudication
  • sometime Flu-like symptoms w/ joint and muscular pain
  • elevated ESR
A

Temporal (Giant cell) Arteritis

  • associated w/ polymyalgia rheumatica
  • Tx: Steroids (STAT)
29
Q

Microscopic finding associated w/ Wegner’s

A

Desrtuctive leukocytic Angitis of arteries an veins

- neutrophils, plasma cells, and eospinophils surrounding necrotic granuloma

30
Q

Telangiectasia (definition)

A

Permanent dilation of preexisting small vessels (usually in skin or mucous membranes

  • can be congenital or acquired
  • not true neoplasm
31
Q

Juvenile (strawberry) Hemangiomas (characteristics)

A

a form of Capillary hemangioma

  • usually found on newborn skin
  • grow rapidly for a few months then fade by 1-3 years
32
Q

AGEs effects on vasculature:

A
  1. Induce cross-linking of collagen which can cause vascular stiffening and entrapment of LDL in the artery walls
  2. AGEs can oxidize LDL (major atherosclerosis developmental factor)
  3. AGEs bind to their receptors (RAGE) and cause oxidative stress as well as activate inflammation pathways causing further injury
33
Q

Difference between Wegner’s and Microscopic polyarteritis?

A
  • Palpable Purpura
  • MPO-ANCA
  • NO Granulomas!
34
Q

An abdominal aneursym is most likely caused by:

A

Atherosclerosis

-Risk factors: men smokers > 50 yrs old

35
Q

Presentation (triad) for AAA

A
  1. pulsatile abdominal mass
  2. hypotension
  3. flank pain
36
Q

Arteriovenous (AV) Fistulas

A

Congenital anomally characterized by:

  • an abnormal connection of artery to a vein WITHOUT a capillary bed
  • occur most commonly as development defect
  • also occur from ruptured arterial aneurysms into veins, pierce injury of an artery and vein and from inflammatory necrosis into adjecent vessels
37
Q
  • Chronic Sinusitis w/ saddle nose
  • Hemoptysis (coughing blood)
  • Hematuria (renal involvement)
  • C-ANCAs (Proteinase-3)
A

Granulomatosis w/ Polyangitis (Wegner’s)

Triad:

  1. Granulomas
  2. Vasculitis
  3. Glomerulonephritis
38
Q

Name 6 small vessel vasculitis disease

A
  1. Granulomatosis w. Polyangitis (Wegner’s)
  2. Microscopic Polyangitis
  3. Churg-Strauss Sundrome
  4. Behget Disease
  5. Henoch-Schonlein purpura
  6. Cryoglobulinemia
39
Q
  1. Palpable purpura located on legs and ass
  2. Arthritis
  3. Abdominal pain (potentally some bloody stool)
  4. Renal disease (severe cases)

-Intussusception (from inflammed intestines)

A

Henoch-Schonlein Purpura

40
Q

Kaposi’s Sarcoma

A

caused by KSHV (aka Human herpesvirus-8)

  • disrupts normal cell division (prevents apoptosis by inhibiting p53)
  • often found in late stage HIV patients
  • also causes primary effusion lymphoma and castleman’s disease
  • very similar to angiosarcoma
41
Q

Vasculitis associated w/ Hepatitis B

A

Polyarteritis Nodosa

  • Generalized vascular involvement
  • NO lung involvement
42
Q

Pyogenic Granulomas (characteristics)

A

type of capillary hemangiomas

  • grow rapidly in areas of trauma
  • oftern pedunculated
  • often found on skin, gingiva, and oral mucosa
  • *resembles granulation tissue
  • painless and associated w/ pregnancy
43
Q

Churge-Strauss Syndrome is characterized by?

A
  1. Affecting those with allergies or asthma
  2. Some type of organ damage
  3. MPO-ANCA
  4. Myocardial involvment
44
Q

Cystic Hygroma

A

Congenital Lymphangioma

  • often found on neck or axilla of children
  • *associated w/ Turner’s syndrome (45, X)
45
Q

Cavernous Hemangiomas (characteristics)

A
  • Do not regress
  • More infiltrative and intimately involved w/ surrunding tissue
  • large blood filled spaces
  • *associated w/ Von Hippel-Lindau syndrome
46
Q

Pathogenesis of Atherosclerosis

A
  1. Endothelial damage allows lipids (LDL and cholesterol) to enter and accumulate in the intima
  2. Lipids are oxidized and consumed by macrophages forming foam cells
  3. Inflammation and healing leads to deposition of ECM and smooth muscle proliferation
47
Q

Risk Factors for HTN

A
  1. Age
  2. Race (increased in African Americans)
  3. obesity
  4. stress
  5. high-salt intake
48
Q

Cause of Henoch-Schonlein Purpura? Prognosis?

A
  1. IgA-immune complex deposits in the mesangium (capillaries of the glomerulus of kidney)
  2. usually secondary to viral upper respiratory infection (URI)

*Generally benign (good prognosis)

49
Q

Angiosarcomas of the Liver are associated with exposures to:

A
  1. Polyvinyl Chloride (PVC) in plastics
  2. Arsenical pesticides
  3. Thorotrast (radioactive contrast agent)
50
Q

Presentation of dissection

A

sharp tearing chest pain that radiates to back

51
Q

nonobstructive calcification of the media within muscular (medium-sized) arteries

A

Monckeberg’s (medial calcific sclerosis)

*ASYMPTOMATIC

52
Q

Function of endothelial cells:

A
  1. Maintain non-thrombotic state (requires normal laminar flow and BP)
  2. Modulates inflammation
  3. Affect growth of other cells (Smooth muscle cells)
  4. Produce NO
  5. Produce contracting factors (endothelin)
53
Q

Name the main infectious agents that cause vasculitis (Bacterial and Fungal)

A

Bacterial: Pseudomonas

Fungi: Aspergillis and Mucor species

54
Q

Causes of HTN

A
  1. Idiopathic (>90%)
  2. Renal disease
  3. Aortic abnormalities (Coarctation + rigidity)
  4. Acute Stress
  5. Pheochromocytoma
55
Q

Main complication of Dissection

A

Cardiac tamponade

  • Also
  • rupture => fatal hemorrhage
  • obstruction of branching arteries (coronary or renal)
56
Q

Complications of AAA

A

*1. Rupture (risk based on size) - may lead to fatal hemorrhage

  1. embolism
  2. impingement on adjacent structures
57
Q

Vasculitis associated with Hepatitic C?

A

Cyroglobulinemia

58
Q

Most common vasculitis of childeren?

A

Henoch-Schonlein Purpura

59
Q

Hyaline vs hyperplastic arteriolosclerosis?

Histologically

A

Hyaline = thickened intima and narrowed artery

Hyperplastic = fibrinoid necrosis of artery (onion skinning apperance)