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STEP 4: Cardio PATHOMA FA > Vascular Pathology > Flashcards

Vascular Pathology Flashcards

1
Q

What are the 3 pathologic patterns of ARTERIOSCLEROSIS?

A
  1. ATHEROSCLEROSIS
  2. ARTERIOLOSCERLOSIS - “OLO” added
  3. MONCKEBERG MEDIAL SCLEROSIS
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2
Q

What layer of the BV wall is thickened in ATHEROSCLEROSIS? Which sized vessels are most commonly affected?

A

INTIMA

Affects MEDIUM/LARGE sized vessels

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3
Q

What sized BV does ARTERIOLOSCLEROSIS affect?

A

SMALL BV (arteriOLO - Arterioles)

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4
Q

What is MONCKEBERG MEDIAL SCLEROSIS? What sized BV does it affect

A

Calcifications of the MEDIA Layer of the BV (MEDIUM SIZED ARTERIES)

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5
Q

What is the predominant component of the INTIMAL PLAQUE in ATHEROSCLEROSIS?

A

Composed of mainly CHOLESTEROL
Necrotic Lipid Core = Cholesterol + Fibromuscular cap
Often with DYSTROPHIC CALCIFICATIONS

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6
Q

What are the 4 most commonly involved arteries of ATHEROSCLEROSIS?

A

MEDIUM/LARGE BV:
“ICAP” - Atherosclerosis has a fibromuscular CAP
Internal Carotid + Coronary + Abdominal aorta + Popliteal

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7
Q

What are the MODIFIABLE (4) risk factors of ATHEROSCLEROSIS?

A

HTN
HL - LDL increases risk, HDL decreases risk
SMOKING
DIABETES

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8
Q

What are the 3 NON-MODIFIABLE risk factors of ATHEROSCLEROSIS?

A

AGE - Increasing age
GENDER - Males, post-menopausal females
GENETICS - FH is highly predictive of atherosclerosis

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9
Q

**UW: Describe the pathogenesis of ATHEROSCLEROSIS (Minimally raised YELLOW SPOTS on inner surface)

A

STEP 1: DAMAGE to endothelium -> Lipids leak into INTIMA
STEP 2: FOAM CELLS/FATTY STREAK: LDL accumulation -> Lipids are oxidized and consumed by MACROPHAGES via scavenger receptors -> FOAM CELLS -> FATTY STREAKS
STEP 3: FIBROMUSCULAR CAP dvlm resulting in PLAQUE = Inflammation + Healing -> ECM deposition and SM proliferation + T cell recruitment -> COMPLEX ATHEROMAS

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10
Q

ATHEROSCLEROSIS COMPLICATION 1: What complications (3) can result from >70% stenosis due to ATHEROSCLEROTIC PLAQUE?

A
  1. CORONARY ARTERY stenosis -> ANGINA
  2. POPLITEAL ARTERY stenosis -> PERIPHERAL VASCULAR DISEASE
  3. MESENTERIC ARTERY stenosis -> ISCHEMIC BOWEL DISEASE
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11
Q

ATHEROSCLEROSIS COMPLICATION 2: Which complications (2) can result from ATHEROSCLEROSIS PLAQUE RUPTURE + thrombosis?

A
  1. CORONARY ARTERY plaque rupture + transmural thrombosis = MI
  2. MIDDLE CEREBRAL ARTERY plaque rupture + thrombosis = STROKE
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12
Q

ATHEROSCLEROSIS COMPLICATION 3: What is the hallmark of ATHEROSCLEROTIC EMBOLI?

A

CHOLESTEROL CLEFTS in embolus that dislodges from the plaque

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13
Q

ATHEROSCLEROSIS COMPLICATION 4: Describe the pathophysiology of how an ATHEROSCLEROTIC PLAQUE can result in an ANEURYSM (Eg. ABDOMINAL AORTA ANEURYSM).

A

ATHEROSCLEROTIC PLAQUE of intimal wall -> Blood carrying oxygen has a HARDER time diffusing across intima to media to adventitia -> BV wall (3 layers of live tissue) gets DEPRIVED of Oxygen -> Wall weakens -> Increases risk of ANEURYSM

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14
Q

What are the 2 types of ARTERIOLOSCLEROSIS?

A
  1. HYALINE

2. HYPERPLASTIC

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15
Q

What is visible upon microscopy of HYALINE ARTERIOSCLEROSIS?

A

Pink hyaline thickening of vascular wall (proteins leaking into BV wall)

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16
Q

***What are the 2 most common causes of HYALINE ARTERIOSCLEROSIS?

A

BENIGN HTN - High pressure pushes proteins into wall

DIABETES - Nonenzymatic glycosylation of BM -> Leaky BV -> Protein leaks in -> Hyaline arteriolosclerosis

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17
Q

Describe the pathophysiology of how ARTERIOLOSCLEROSIS can progress to CHRONIC RENAL FAILURE.

A

ARTERIOSCLEROSIS -> Protein leakage into BV wall and thickening -> REDUCED caliber of RENAL AFFERENT ARTERIOLE -> End-organ [kidney] ischemia -> GLOMERULAR SCARRING = ARTERIOLONEPHROSCLEROSIS -> Can ultimately progress to CHRONIC RENAL FAILURE

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18
Q

Describe what is seen in the gross appearance of a kidney that has been affected by ARTERIOLOSCLEROSIS (Renal afferent arteriole).

A

Shrunken kidneys - Due to arterionephrosclerosis (glomerular scarring)
Scarring on surface of kidney (CORTEX)

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19
Q

ONION SKIN APPEARANCE of a BV

A

HYPERPLASTIC ARTERIOLOSCLEROSIS - Due to Smooth muscle hyperplasia decreasing the blood flow to end organ

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20
Q

What is the most common cause of HYPERPLASTIC ARTERIOLOSCLEROSIS?

A

MALIGNANT HTN: Super high bp -> SM responds by excessive proliferation in attempt to CONTAIN that high BP

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21
Q

What are two pathologies that you see FIBRINOID NECROSIS (=death of vessel wall)?

A

MALIGNANT HTN + VASCULITIS

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22
Q

**What is the GROSS APPEARANCE of the kidney that has been affected by HYPERPLASTIC ARTERIOLOSCLEROSIS?

A

**FLEA BITTEN KIDNEY - Due to ACUTE RENAL FAILURE that precipitated from reduced vessel caliber with end-organ ischemia -> Pin point hemorrhages on the surface of kidney

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23
Q

FIBRINOID NECROSIS (death of BV wall) + FLEA BITTEN SURFACE OF KIDNEY = what type of arteriosclerosis? What is the most common cause?

A

HYPERPLASTIC ARTERIOLOSCLEROSIS

Most common cause = MALIGNANT HTN

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24
Q

How is MONCKEBERG MEDIAL CALCIFIC SCLEROSIS generally discovered?

A

NON-OBSTRUCTIVE as in Atherosclerosis or arteriolosclerosis - Therefore NOT clinically significant
Detected as INCIDENTAL finding on X-RAY or MAMMOGRAPHY

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25
Q

PIPE STEM APPEARANCE of CALCIFICATIONS ON X-RAY in the pattern of the vessels

A

MONCKEBERG MEDIAL CALCIFIC SCLEROSIS

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26
Q

What layers of the BV wall are affected by an AORTIC DISSECTION?

A

TEAR through the Media INTIMA

BLOOD DISSECTS through media of aortic wall

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27
Q

What are the 2 requirements of an AORTIC DISSECTION?

A
  1. HIGH STRESS: Occurs at the proximal 10cm of the aorta (ARCH or ASCENDING AORTA)
  2. PRE-EXISTING WEAKNESS OF TUNICA MEDIA: HTN is most common cause of media weakness
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28
Q

How does HTN result in WEAKNESS of TUNICA MEDIA (1 of 2 requirements of AORTIC DISSECTION)?

A

HTN -> Hyaline arteriolosclerosis of VASO VASORUM supplying the outer half of the BV wall -> Reduced luminal caliber -> Decrease blood flow to outer half -> SMOOTH MUSCLE (MEDIA) ATROPHY = weakness

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29
Q

What are 2 other less common congenital causes of that result in WEAKNESS of TUNICA MEDIA (1 of 2 requirements of AORTIC ANEURYSM)?

A

MARFAN SYNDROME

EHLER DANLOS SYNDROME

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30
Q

Cardiac Pathologies associated with MARFAN [defect in fibrillin]/EHLER DANLOS SYNDROME [defect in collagen]

A
  1. MVP
  2. CYSTIC MEDIAL AORTIC DEGENERATION -> AORTIC DISSECTION (Tunica media contains HIGH amounts of ELASTIC connective tissue)
  3. THORACIC AORTA ANEURYSM (Weakness of entire BV wall -> Predisposes to dvlm of aneurysm)
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31
Q

What is the most common cause of death in pts with an AORTIC DISSECTION?

A

PERICARDIAL TAMPONADE

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32
Q

What are the 3 complications of an AORTIC DISSECTION?

A
  1. END ORGAN ISCHEMIA (KIDNEY - acute renal failure): Blood from aneurysm flows FORWARD and compresses onto the renal artery
  2. PERICARDIAL TAMPONADE: Blood from aneurysm flows BACKWARD and compresses the heart
  3. Rupture with FATAL HEMORRHAGE into MEDIASTINUM
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33
Q

What is the ONE requirement of an ANEURYSM?

A

Weakness in the AORTIC WALL

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34
Q

What is the classic cause of THORACIC AORTIC ANEURYSM. Describe the pathophysiology.

A

TERTIARY SYPHILIS
Endarteritis of VASOVASORUM -> Reduced luminal caliber -> Decreased blood flow -> Atrophy of BV wall -> WEAKNESS of BV WALL (1 of 1 requirement of an aneurysm)

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35
Q

What is the most common complication of a THORACIC AORTA ANEURYSM? What are the 2 less common complications?

A
  1. AORTIC REGURGITATION**: Due to Aneurysm creating an aortic root dilation -> Stretches the aortic valve -> Insufficiency
  2. DYSPHAGIA or DYSPNEA: Due to compression of mediastinal structures (Airway, esophagus)
  3. THROMBUS/EMBOLUS: Balloon-like dilation -> Turbulent flow instead of laminar flow -> Activates COAG cascade
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36
Q

TREE BARK APPEARANCE OF AORTA is associated with which pathology?

A

TERTIARY SYPHILIS resulting in endarteritis of vasovasorum -> Scarring/fibrosis of BV wall -> THORACIC AORTA ANEURYSM -> Aortic regurgitation
Tree bark = Scarring and fibrosis of aorta

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37
Q

Where is the most common location of an ABDOMINAL AORTA ANEURSYM?

A

Distal to RENAL ARTERIES, proximal to ABDOMINAL AORTA BIFURCATION

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38
Q

What is the most common cause of AAA? Who is the typical, classical pt?

A

ATHEROSCLEROSIS

Typical pt = risk factors of atherosclerosis (MALE SMOKER >60yo with HTN, HL)

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39
Q

When the pulsatile abdominal mass of an ABDOMINAL AORTIC ANEURYSM grows with time, it eventually becomes >5cm in diameter. What is the main complication and clinical presentation at this point?

A

FEAR OF ANEURYSM RUPTURE
Clinical Presentation = 1. HYPOTENSION (Rupture resulting in bleeding and shock) + 2. PULSATILE MASS (Feel blood coursing through) + 3. FLANK PAIN

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40
Q

Infant presents with a BENIGN TUMOR comprised of BV on the face. What is the Tx of choice for this pt?

A

Pt has a HEMANGIOMA. NO SURGICAL resection that would result in a scar bec it often regresses during childhood.

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41
Q

What is another common location of a HEMANGIOMA?

A

LIVER + SKIN

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42
Q

How does one differentiate between a HEMANGIOMA of the skin and a PURPURA.

A

Pressing down on the HEMANGIOMA: YES BLANCHING because it is a tumor of the BV
Pressing down on the PURPURA: NO BLANCHING because there is actual bleeding in the skin

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43
Q

What is a malignant proliferation of ENDOTHELIAL CELLS that is HIGHLY AGGRESSIVE?

A

ANGIOSARCOMA

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44
Q

What tissues does a HEMANGIOSARCOMA most commonly involve?

A

SKIN
LIVER
BREAST

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45
Q

What are 3 RISK FACTORS of a LIVER ANGIOSARCOMA?

A

POLYVINYL CHLORIDE
ARSENIC
THOROTRAST

46
Q

Would pressing on a KAPOSI SARCOMA result in BLANCHING?

A

NO, would NOT result in blanching (as in a HEMANGIOMA)

Because it is a proliferation of ENDOTHELIAL CELLS that are NOT BV yet (Blood is simply interspersed within channels of skin)

47
Q

Which virus drives KAPOSI SARCOMA (Low grade malignant tumor proliferation of endothelial cells)? Which 3 pt populations does it most commonly affect?

A

HHV-8

48
Q

What is a malignant proliferation of ENDOTHELIAL CELLS that is HIGHLY AGGRESSIVE?

A

ANGIOSARCOMA

49
Q

What tissues does a HEMANGIOSARCOMA most commonly involve?

A

SKIN
LIVER
BREAST

50
Q

What are 3 RISK FACTORS of a LIVER ANGIOSARCOMA?

A

POLYVINYL CHLORIDE
ARSENIC
THOROTRAST

51
Q

Would pressing on a KAPOSI SARCOMA result in BLANCHING?

A

NO, would NOT result in blanching (as in a HEMANGIOMA)

Because it is a proliferation of ENDOTHELIAL CELLS that are NOT BV yet (Blood is simply interspersed within channels of skin)

52
Q

Which virus drives KAPOSI SARCOMA (Low grade malignant tumor proliferation of endothelial cells)? Which 3 pt populations does it most commonly affect?

A

HHV-8
3 pt populations -
1. OLDER EASTERN EUROPEAN MALES: Tx = surgical removal of tumor localized to skin
2. AIDS: Tx = anti-retroviral agents (To boost immune system and increase CD4 ct)
3. Tx Recipients: Tx = decrease immunosuppression

53
Q

What are the 2 possible clinical presentations of VASCULITIS?

A
  1. NON-SPECIFIC Sx of inflammation (fever, weight loss, myalgia)
  2. END-ORGAN ISCHEMIA: Vasculitis -> Inflammation and damage of endothelial cells -> Tissue fibrosis -> LUMINAL NARROWING + THROMBOSIS (due to exposed sub-endothelial collagen)
54
Q

What are the 2 types of LARGE VESSEL VASCULITISES?

A
  1. TEMPORAL (GIANT CELL) ARTERITIS

2. TAKAYASU ARTERITIS

55
Q

GIANT CELL ARTERITIS: Who is the classic typical pt affected by GIANT CELL ARTERITIS?

A

FEMALE age>50yo

56
Q

GIANT CELL ARTERITIS: Which vessels can possibly be inflamed in GIANT CELL TEMPORAL ARTERITIS? What are the associated clinical sx with each vessel?

A

Branches of the CAROTID ARTERY

  1. TEMPORAL ARTERY - Headache
  2. OPHTHALMIC ARTERY - Visual disturbances
  3. ARTERIES SUPPLYING JAW MUSCLES - Jaw claudication
57
Q

GIANT CELL ARTERITIS: What is POLYMYALGIA RHEUMATICA?

A

Pts with GIANT CELL ARTERITIS who have FLU-like sx + MYALGIA

NECK, TORSO, SHOULDER, PELVIC GIRDLE PAIN + MORNING STIFFNESS

58
Q

GIANT CELL ARTERITIS: What is the marker of inflammation with GIANT CELL ARTERITIS?

A

ESR >150

59
Q

GIANT CELL ARTERITIS: What is seen on BIOPSY of a GIANT CELL TEMPORAL ARTERITIS?

A
  1. GRANULOMATOUS INFLAMMATION: Giant cells + Epithelioid histiocytes [macrophages] + Lymphocytes
  2. INTIMAL FIBROSIS (post-inflammation healing)
60
Q

To diagnose GIANT CELL ARTERITIS, is it safe to look at one portion of the vessel? If the biopsy comes out negative, can I r/o GIANT CELL ARTERITIS?

A

It is NOT safe to look at only portion of the vessel. Lesions of GIANT CELL ARTERITIS are SEGMENTAL - 1) Need to take out a LONG segment of the vessel and 2) Need to look at ALL parts of the vessel under the microscope

NO, can NOT rule out with a negative biopsy bec it just means that the segment you might have taken out for biopsy doesn’t contain the lesion

61
Q

If GIANT CELL ARTERITIS is suspected, what is the IMMEDIATE TREATMENT (even before diagnosis is confirmed)? What is the reason?

A

CORTICOSTEROIDS immediately

Due to high risk of BLINDNESS without Tx (endothelial damage of ophthalmic artery is irreversible)

62
Q

TAKAYASU ARTERITIS: Who is the typical pt affected?

A

Unlike GIANT CELL TEMPORAL ARTERITIS (>50yo females), TAKAYASU

63
Q

TAKAYASU ARTERITIS: What is seen on biopsy of TAKAYASU ARTERITIS?

A

Granulomatous vasculitis - Same as GIANT CELL ARTERITIS [epithelioid histiocytes + giant cells + lymphocytic rim]

64
Q

TAKAYASU ARTERITIS: What vessel is affected?

A

AORTA ARCH at branch points

65
Q

TAKAYASU ARTERITIS: How do you differentiate TAKAYASU ARTERITIS with GIANT CELL ARTERITIS (2)?

A

TAKAYASU : 50yo females, 2. Affects branches of carotid artery (temporal, ophthalmic, jaw muscles)

66
Q

TAKAYASU ARTERITIS: Why is TAKAYASU ARTERITIS sometimes called PULSELESS DISEASE?

A

Because of UE Claudication (Weak/Absent pulse)

67
Q

TAKAYASU ARTERITIS: Similar to GIANT CELL ARTERITIS, what is the clinical presentation of TAKAYASU ARTERITIS? What is the inflammatory marker? What is the Tx?

A

CLINICAL PRESENTATION: Visual, neurologic deficits, UE claudication + Non-specific systemic signs (fever, myalgia, headaches)
ESR >150
Tx = CORTICOSTEROIDS

68
Q

What arteries are involved in MEDIUM-VESSEL VASCULITIS?

A

MUSCULAR ARTERIES (Eg. Renal artery)

69
Q

Name the 3 types of MEDIUM VESSEL VASCULITIS.

A
  1. POLYARTERITIS NODOSA
  2. KAWASAKI
  3. BUERGER
70
Q

POLYARTERITIS NODOSA: Which MUSCULAR arteries are involved with PAN and what are the associated Sx?

A

RENAL ARTERY: HTN
MESENTERIC ARTERY: Abdominal Pain + Melena
INTERNAL CAROTID ARTERY: Neurologic deficits
+ Skin lesions

71
Q

POLYARTERITIS NODOSA: All organs can basically be involved with PAN, except for which organ?

A

LUNGS

72
Q

POLYARTERITIS NODOSA: Which infection is most commonly associated with PAN? How can this be confirmed?

A

HEPATITIS B

+HBsAg

73
Q

POLYARTERITIS NODOSA: What is the histological change on biopsy associated with PAN? Which other pathology do you also see this process?

A

FIBRINOID NECROSIS: Lots of PINK in the BV wall

Also seen with MALIGNANT HTN resulting in HYPERPLASTIC ARTERIOLOSCEROSIS

74
Q

STRING OF PEARLS APPEARANCE ON IMAGING (that is NOT FIBROMUSCULAR DYSPLASIA). Why do you this string of pearls?

A

POLYARTERITIS NODOSA - See string of pearls based on stage of lesion
EARLY STAGE LESION (STRINGS)= Transmural inflammation + Fibrinoid necrosis
LATE STAGE LESION (PEARLS) = Post-inflammation healing with fibrosis = NODULES/NODES on arteries

75
Q

POLYARTERITIS NODOSA: What complication do you have an increased risk of due to the fibrotic nodules that form on the affected muscular arteries?

A

WEAKENED BV WALL -> Increased risk of ANEURYSMS

76
Q

POLYARTERITIS NODOSA: What is the Tx (2) of POLYARTERITIS NODOSA?

A

CORTICOSTEROIDS + CYCLOPHOSPHAMIDE

77
Q

KAWASAKI DISEASE: Who is the typical pt affected with KAWASAKI disease?

A

Asian child

78
Q

KAWASAKI DISEASE: Think of a kid on a motorcycle putting hands on handles, and HR racing. What is the typical presentation?

A 
VERY NON-SPECIFIC: Almost seems like a VIRAL infection 
FEVER
CONJUNCTIVITIS 
ERYTHEMATOUS RASH ON PALMS AND SOLES 
ENLARGED CERVICAL LYMPH NODES
79
Q

KAWASAKI DISEASE: What is the most commonly involved artery? What are the two possible complications?

A

CORONARY ARTERY
COMPLICATION 1: MI - Vasculitis of coronary artery -> Exposure of subenodethlial collagen -> Activation of coagulation cascade -> Transmural ischemia due to thrombus
COMPLICATION 2: ANEURYSM with rupture - Due to weakened BV wall post-vasculitis

80
Q

When a baby presents with viral infection-like symptoms what is the Tx that you must absolutely avoid? To prevent which pathology?

A

ASA

To prevent REYES SYNDROME

81
Q

KAWASAKI DISEASE: What is the Tx? What is the mechanism?

A

1 .ASA - Selective inhibition of COX-1 -> Selective decreases of TXA2 (normally facilitating PLT aggregation) -> Decreases thrombus formation in coronary artery
2. IVIG

82
Q

BUERGER DISEASE: What is the most common association with BUERGER DISEASE? Thus what is the Tx?

A

SMOKING - Highest association with BUERGER DISEASE (Medium vessel vasculitis)
Tx = SMOKING CESSATION

83
Q

BUERGER DISEASE: Which organs are most commonly involved?

A

NECROTIZING VASCULITIS of the DIGITS

84
Q

BUERGER DISEASE: What is the typical clinical presentation of BUERGER DISEASE?

A

ULCERATION + GANGRENE + AUTO-AMPUTATION OF FINGERS/TOES + RAYNAUD

85
Q

What types of vessels are affected with SMALL VESSEL VASCULITIS? Name the 4 types of SMALL VESSEL VASCULITIS.

A

ARTERIOLES, CAPILLARIES, VENULES

4 types: WEGENER GRANULOMATOSIS, MICROSCOPIC POLYANGIITIS, CHURG-STRAUSS SYNDROME, HENOCH-SCHONLEIN PURPURA

86
Q

WEGENER GRANULOMATOSIS: Think WECENER GRANULOMATOSIS - What are the 3 most commonly involved organs? Name the associated Sx

A

NASOPHARYNX - SINUSITIS + NASOPHARYNGEAL ULCERATION
LUNG - Hemoptysis + Bilateral Lung Nodular Infiltrates
KIDNEYS - Hematuria due to RPGN (rapid progressive glomerulonephritis)

87
Q

WEGENER GRANULOMATOSIS: Who is the typical pt affected?

A

MIDDLE AGED MALES

88
Q

WEGENER GRANULOMATOSIS: What is the typical marker that correlates with disease activity?

A

C-ANCA

THINK “C”

89
Q

WEGENER GRANULOMATOSIS: What is the Tx?

A

Think C

CYCLOPHOSPHAMIDE + CORTICOSTEROIDS

90
Q

WEGENER GRANULOMATOSIS: What is the classic histological finding on biopsy?

A

NECROTIZING GRANULOMAS + Surrounding NECROTIZING VASULITIS

91
Q

MICROSCOPIC POLYANGIITIS: Which organs does Microscopic polyangiitis preferentially involve?

A

INVOLVES MULTIPLE ORGANS, but particularly affects LUNG + KIDNEY

92
Q

MICROSCOPIC POLYANGIITIS: Is similar to WEGENER GRAULOMATOSIS with 3 exceptions
[Hint: 1 clinical sx, 1 biopsy finding, 1 diagnostic test finding]

A

MICROSCOPIC POLYANGIITIS: NO nasopharyngeal involvement (sinusitis/nasopharyngeal ulcers), NO GRANULOMAS, P-ANCA correlating with disease activity

WEGENER: YES nasopharyngeal involvement, YES granulomas, c-ANCA not p-ANCA correlating with disease activity

93
Q

What is the Tx of MICROSCOPIC POLYANGIITIS? Are relapses common or uncommon?

A

Same as WEGENER GRANULOMATOSIS - CORTICOSTEROIDS + CYCLOPHOSPHAMIDE, relapses are common

94
Q

CHURG-STRAUSS SYNDROME: What is the common diagnostic test finding between MICROSCOPIC POLYANGIITIS and CHURG-STRAUSS?

A

p-ANCA

95
Q

CHURG-STRAUSS SYNDROME: Name 3 distinguishing features between CHURG-STRAUSS and MICROSCOPIC POLYANGIITIS.
[Hint: 1 PMH key note, 2 biopsy findings]

A
  1. CHURG-STRAUSS: History of ASTHMA, whereas microscopic polyangiitis does NOT
  2. CHURG-STRAUSS: YES granulomas (MPA - no granulomas) + Peripheral EOSINOPHILIA (MPA - no eosinophils)
96
Q

What is the most common vasculitis in children?

A

HENOCH SCHONLEIN PURPURA (HSP) in children = SMALL VESSEL VASCULITIS

97
Q

HSP: What is the pathophysiology of HSP? What infection does this commonly follow?

A

IgA deposits

UPPER RESPIRATORY INFECTION

98
Q

HENOCH SCHONLEIN PURPURA (HSP): What are the 3 typical presentations of HSP? Which is the most likely to occur?

A
  1. IgA NEPHROPATHY = most likely - Presents with HEMATURIA
  2. PALPABLE purpura along buttocks and legs - PALPABLE (other purpuras are NOT palpable, whereas HSP ones are due to the INFLAMMATION along the vasculitis)
  3. GI PAIN + BLEEDING
99
Q

**UWorld: What is the most common cause of death in MARFAN SYNDROME pts? What is the second most common cause of death?

A

AORTA DISSECTION = most common cause

Heart failure secondary to MVP or Aortic regurgitation

100
Q

**UWorld: Segmental vasculitis EXTENDING INTO contiguous veins and nerves is characteristic and unique to which vasculitis?

A

BUERGER’S DISEASE

101
Q

**UWorld: Lipid intimal plaques that bulge into the arterial lumen is characteristic of what pathology?

A

ATHEROSCLEROSIS

102
Q

**UWorld: Onion-like concentric thickening of arteriolar walls as a reuslt of LAMINATED SMC and reduplicated BM is characteristic of what pathology?

A

HYPERPLASTIC ARTERIOLOSCERLOSIS (From MALIGNANT HTN)

Malignant HTN = DIASTOLIC PRESSURES >120-130
Presents with HEADACHE, RENAL FAILURE, PAPILLEDEMA (medical emergency)

103
Q

**UWorld: Granulomatous inflammation of the MEDIA is characteristic of what pathology?

A

TERMPORAL (GIANT CELL) ARTERITIS = most common form

OTHERS: TAKAYASU
WEGNER’S, CHURG-STRAUSS

104
Q

**UWorld: Fibrinoid necrosis (Death of BV) = Which 2 pathologies?

Fibrinoid Necrosis + TRANSMURAL INFLAMMATION of arterial wall = Which of the 2?

A
  1. HYPERPLASTIC ARTERIOLOSCLEROSIS (malignant HTN Diastolic BP>120-130)
  2. POLYARTERITIS NODOSA - Fibrinoid necrosis + Transmural inflammation of arterial wall = STRING OF PEARLS
105
Q

**UW: Large FRIABLE vegetations on valve cusps + DESTRUCTION = ?

A

INFECTIVE ENDOCARDITIS

106
Q

**UW: Diffuse FIBROUS THICKENING + Distortion of leaflets + COMMISSURAL FUSION at leaflet edges + Narrowing of orifice = ?

A

CHRONIC RHEUMATIC FEVER

107
Q

**UW: Which vasculitis has most common association with an MI and/or coronary aneurysm?

A

KAWASAKI

108
Q

**UW: Describe the pathogenesis of ATHEROSCLEROSIS (Minimally raised YELLOW SPOTS on inner surface)

A

STEP 1: DAMAGE to endothelium -> Lipids leak into INTIMA
STEP 2: FOAM CELLS/FATTY STREAK: LDL accumulation -> Lipids are oxidized and consumed by MACROPHAGES via scavenger receptors -> FOAM CELLS -> FATTY STREAKS
STEP 3: FIBROMUSCULAR CAP dvlm resulting in PLAQUE = Inflammation + Healing -> ECM deposition and SM proliferation + T cell recruitment -> COMPLEX ATHEROMAS

109
Q

**UW: High fevers, strawberry tongue, perioral erythema/fissuring, periungual desquamation, swollen lymph nodes, red eyes

A

KAWASAKI DISEASE

110
Q

**UW: TRIAD: JOINT PAIN + LE/BUTTOCK purpura + HEMATURIA

+ Occasional abdominal pain

A

HSP
IgA-mediated HSR vasculitis
Renal pathology is same as IgA NEPHROPATHY (BERGER) = MESANGIAL proliferation + CRESCENT formation

HSP = PALPABLE PURPURA + RENAL DISEASE (HEMATURIA) + ARTHRALGIA/ARTHRITIS

111
Q

**UW: Fibrinoid necrosis (3 pathologies)

A

MALIGNANT HTN + VASCULITIS + SERUM SICKNESS (e.g. mAb treatments + Igs - Type III HSR)

STEP 4: Cardio PATHOMA FA (6 decks)

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