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M3 Path Block 2 > 8 - Blood Vessels: Non-Infectious Vasculitis > Flashcards

8 - Blood Vessels: Non-Infectious Vasculitis Flashcards

0
Q
  1. Giant Cell Arteritis (Temporal Arteritis): Defined
A

Most common type in elderly

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1
Q

Non Infectious Vasculitis: Mechanisms (3) and Types (8)

A

Mechanisms:

  1. Immune Complex Deposition
  2. Anti-neutrophil cytoplasmic antibodies
  3. Anti-endothelial cell antibodies

Types:

  1. Giant Cell Arteritis (GCA)
  2. Takayasu Arteritis
  3. Polyarteritis Nodosa
  4. Kawasaki Disease
  5. Microscopic Polyangitis
  6. Wegener Granulomatosis
  7. Thromboangiitis Obliterans (Buerger)
  8. Vasculitis with other disorders
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2
Q
  1. Giant Cell Arteritis (Temporal Arteritis): Site of Involvement (2)
A
  1. Affects large to small arteries

2. Usually branches of carotid artery (temporal & extra-cranial branches)

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3
Q
  1. Giant Cell Arteritis (Temporal Arteritis): Distribution of Disease
A
  1. Temporal, facial, ophthalmic arteries
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4
Q
  1. Giant Cell Arteritis (Temporal Arteritis): Clinical Features (3)
A
  1. *Throbbing unilateral headache
  2. *Facial Pain
  3. *Visual disturbances (Ophthalmic artery occlusion) –> first diplopia, later on blindness can occur
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5
Q
  1. Giant Cell Arteritis (Temporal Arteritis): Morphology (6)
A
  1. *Nodular Thickenings with reduction of lumen
  2. Segmental *granulomtous (= giant cell) vasculitis with multinucleate giant cells
  3. Fragmentation of internal elastic lamina
  4. Intimal fibrosis with luminal narrowing
  5. *High ESR
  6. *Temporal

Slide 52

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6
Q
  1. Giant Cell Arteritis (Temporal Arteritis): Treatment
A
  1. Corticosteroids
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7
Q
  1. Giant Cell Arteritis (Temporal Arteritis): Epidemiology (3)
A

*Females > Males

Affects elderly (*> 50 years)

*Associated with HLA-Class II and polymyalgia rheumatica

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8
Q
  1. Takayasu Arteritis (Pulseless Disease): Distribution of Disease (2)
A
  1. Medium sized to large arteries

2. Aortic arch

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9
Q
  1. Takayasu Arteritis (Pulseless Disease): Etiology
A

Unknown

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10
Q
  1. Takayasu Arteritis (Pulseless Disease): Gross
A
  1. *Narrowing of orifices
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11
Q
  1. Takayasu Arteritis (Pulseless Disease): Microscopy
A
  1. Granulomatous vasculitis
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12
Q
  1. Takayasu Arteritis (Pulseless Disease): Clinical Presentation
A

Weaker pulses in UL

- Coldness or numbness of fingers

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13
Q
  1. Takayasu Arteritis (Pulseless Disease): Sequelae (2)
A
  1. *Aortic valve insufficiency
    • Due to involvement of aortic root leading to dilation
  2. MI
    • Due to narrowing of the coronary ostia
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14
Q
  1. Takayasu Arteritis (Pulseless Disease): Defined (2)
A
  1. Ocular Disturbances

2. Marked weakening of pulses in upper extremities

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15
Q
  1. Takayasu Arteritis (Pulseless Disease): Epidemiology (2)
A
  1. Asia

2. Young and middle-aged WOMEN (15-45 years)

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16
Q
  1. Polyarteritis Nodosa (PAN): Other Manifestations
A
  1. Lesions are in different stages of development (acute or healing stage)
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17
Q
  1. Polyarteritis Nodosa (PAN): Symptoms (2)
A
  1. *Hematuria, renal failure, HTN

2. *Abdominal pain, diarrhea, GI bleeding

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18
Q
  1. Kawasaki Disease: Pathology (1)
A
  1. Acute *necrotizing vasculitis of small/medium sized vessels
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19
Q
  1. Takayasu Arteritis (Pulseless Disease): Pathology (2)
A
  1. Vasculitis

2. Transmural thickening of aorta with narrowing of lumina

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20
Q
  1. Kawasaki Disease: Clinical Features (3)
A
  1. *Conjunctivitis; erythema and erosions of oral mucosa
  2. *Skin rash - generalized maculopapular
  3. *Lymphadenopathy
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21
Q
  1. Kawasaki Disease: Pathology (2)
A
  1. Acute *necrotizing vasculitis of small/medium sized vessels
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22
Q
  1. Kawasaki Disease: Complications
A

MI

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23
Q
  1. Kawasaki Disease: Prognosis (2)
A
  1. Self-limited course

2. Mortality rate: 1-2% due to rupture of a coronary aneurysm or coronary thrombosis

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24
Q
  1. Microscopic Polyangitis (Leukocytoclastic Vasculitis): Organs Affected
A
  1. Post-capillary venules: mostly skin –> palpable purpura
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25
Q
  1. Takayasu Arteritis (Pulseless Disease): Variable Course (2)
A

Rapid progression

Quiescent stage with 1-2 years

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26
Q
  1. Polyarteritis Nodosa (PAN): Defined
A

Necrotizing immune vasculitis

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27
Q
  1. Polyarteritis Nodosa (PAN): Epidemiology (2)
A

Young adults

M>F

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28
Q
  1. Polyarteritis Nodosa (PAN): Distribution of Disease (2)
A
  1. Systemic necrotizing vasculitis (fibrinoid)
    • Any organ *EXCEPT LUNG
    • *Kidney, *GIT, heart liver
  2. Small and medium sized muscular arteries
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29
Q
  1. Polyarteritis Nodosa (PAN): Cause
A

Immune complex deposition (type III hypersensitivity), how is unknown

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30
Q
  1. Polyarteritis Nodosa (PAN): Associated conditions (2)
A
  1. HBV antigenemia (30-50%)

2. Hypersensitivity to drugs (IV Amphetamines)

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31
Q
  1. Polyarteritis Nodosa (PAN): Other Manifestations (3)
A
  1. Lesions are in different stages of development (acute or healing stage)
  2. Frequently only part of vessel is involved
  3. Nodosa - focal aneurysm formation
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32
Q
  1. Polyarteritis Nodosa (PAN): Symptoms (4)
A
  1. *Hematuria, renal failure, HTN
  2. *Abdominal pain, diarrhea, GI bleeding
  3. Fever, weight loss, malaise
  4. Myalgia and arthralgia
33
Q
  1. Wegeners’s Granulomatosis: Microscopy
A
  1. *Granulomas in Wegner’s
34
Q
  1. Polyarteritis Nodosa (PAN): Signs (2)
A
  1. Micro-aneurysms - weakened channels

2. Pericarditis, myocarditis, palpable purpura

35
Q
  1. Polyarteritis Nodosa (PAN): Segmental Necrotizing Vasculitis Lesions Stages (3 Stages)
A

Acute Lesions - Fibrinoid necrosis and neutrophils

Healing Lesions - Fibroblast proliferation

Healed Lesions - Nodular fibrosis and loss of internal elastic lamina

36
Q
  1. Buerger’s Disease (Thromboangitis Obliterans) : Age
A

Age: 25-50 years

37
Q
  1. Polyarteritis Nodosa (PAN): Complications (2)
A
  1. *Thrombosis and infarction

2. *Multiple aneurysms (kidneys, heart, GIT)

38
Q
  1. Buerger’s Disease (Thromboangitis Obliterans) : Clinical Features (1)
A
  1. Intermittent Claudication
39
Q
  1. Kawasaki Disease: Epidemiology
A

Infants and young children

40
Q
  1. Kawasaki Disease: Distribution of Disease (2)
A
  • Large, medium-sized, and small arteries

* Coronary artery commonly affected

41
Q
  1. Kawasaki Disease: Etiology
A

*Possibly viral

42
Q
  1. Kawasaki Disease: Clinical Features (4)
A
  1. *Conjunctivitis; erythema and erosions of oral mucosa
  2. *Skin rash - generalized maculopapular
  3. *Lymphadenopathy
  4. Fever
43
Q
  1. Vasculitis with Other Disorders: Raynaud’s Phenomenon & Disease - Clinical Features (4)
A
  1. Young woman
  2. *Episodic small artery vasospasm in extremities, nose, or ears
  3. Blanching and cyanosis of fingers or toes
  4. *No under underlying disease or pathology
44
Q
  1. Kawasaki Disease: Pathology (2)
A
  1. Acute *necrotizing vasculitis of small/medium sized vessels
  2. Weakened vascular wall may undergo aneurysm
45
Q
  1. Kawasaki Disease: Complications (3)
A
  1. MI
  2. Thrombosis
  3. Aneurysm
46
Q
  1. Kawasaki Disease: Prognosis (2)
A
  1. Self-limited course

2. Mortality rate: 1-2% due to rupture of a coronary aneurysm or coronary thrombosis

47
Q
  1. Microscopic Polyangitis (Leukocytoclastic Vasculitis): Organs Affected (2)
A
  1. Post-capillary venules: mostly skin –> palpable purpura

2. Can affect internal organs and tissues

48
Q
  1. Microscopic Polyangitis (Leukocytoclastic Vasculitis): Pathogenesis (2)
A
  1. *Immune Complex deposition (Type III Hypersensitivity)

2. **P-ANCA positive (Myeloperoxidase-ANCA)

49
Q
  1. Microscopic Polyangitis (Leukocytoclastic Vasculitis): Causes (2)
A
  1. Drugs: Penicillin, sulfonamides (haptens)

2. Bacterial: Beta hemolytic Streptococcal Ag, Hep B Ag

50
Q
  1. Microscopic Polyangitis (Leukocytoclastic Vasculitis): C/F (4)
A
  1. Hemoptysis
  2. Hematuria
  3. Proteinuria
  4. Cutaneous purpura
51
Q
  1. Microscopic Polyangitis (Leukocytoclastic Vasculitis): Microscopically
A

*Nuclear dust from neutrophil breakdown (Slide 67)

52
Q
  1. Churg-Strauss Syndrome: Defined and Association (8)
A

Aka: allergic granulomatosis and angiitis

Obscure etiology

  • SMALL VESSEL necrotizing vasculitis classically associated with
    1. Asthma
    2. Allergic rhinitis
    3. Lung infiltrates
    4. Peripheral hyper-eosinophilia
    5. Extravascular necrotizing GRANULOMAS
53
Q
  1. Churg-Strauss Syndrome: Microscopic Appearance
A

*Characteristically accompanied by granulomas and eosinophils

54
Q
  1. Churg-Strauss Syndrome: Diagnosis
A

*ANCAs (Mostly MPO-ANCAS) are present

55
Q
  1. Wegener’s Granulomatosis: Epidemiology (3)
A

M>F

Rare

Age: 40-60 peak

56
Q
  1. Wegener’s Granulomatosis: Distribution of Disease (3)
A
  1. *Focal necrotizing vasculitis WITH GRANULOMAS
  2. *Involves nose, sinuses, lungs, and kidneys
  3. Small size arteries & veins
57
Q
  1. Wegener’s Granulomatosis: CNKA is now called?
A

Proteinase 3 (cytoplasm of neutrophils called proteinase 3)

58
Q
  1. Wegener’s Granulomatosis: Classic Triad (3)
A
  1. Acute necrotizing: seen in nose, sinuses, upper airways, and lungs
  2. Granulomatous vasculitis: affects small size arteries and veins in other organs
  3. Necrotizing glomerulonephritis: important cause of RPGN (crescents)
59
Q
  1. Wegener’s Granulomatosis: Clinical Features (4)
A
  1. Kidneys
  2. Chronic sinusitis
  3. Otitis media
  4. Perforation of nasal septum
60
Q
  1. Wegeners’s Granulomatosis: Microscopy (3)
A
  1. *Granulomas in Wegner’s
  2. Neutrophils
  3. Fibrinoid Necrosis
61
Q
  1. Wegener’s Granulomatosis: Lab Findings (2)
A
  1. *C-ANCA (autoantibody against proteinase 3)
    • correlates with disease activity
  2. Urine examination - hematuria
62
Q

Slide 73

A

Lecture Capture to get April 10th’s Summary of Diseases Mentioned!

63
Q
  1. Buerger’s Disease (Thromboangitis Obliterans) : Age and Etiology (2)
A

Age: 25-50 years

Etiology: Cigarette smoking

64
Q
  1. Buerger’s Disease (Thromboangitis Obliterans) : Site (2)
A
  1. Radial and Tibial Arteries

2. Adjavent nerves and veins

65
Q
  1. Buerger’s Disease (Thromboangitis Obliterans) : Clinical Features (4)
A
  1. Intermittent Claudication
  2. Superficial nodular phlebitis
  3. Raynaud’s phenomenon - finger
  4. May lead to distal gangrene
66
Q
  1. Buerger’s Disease (Thromboangitis Obliterans) : Pathology (4)
A
  1. Thrombosis, organization, recanalization
  2. Micro-abscess: neural and vein involvement
  3. Granulomas
  4. Fibrosis of vascular bundle
67
Q
  1. Vasculitis with Other Disorders: Raynaud’s Phenomenon & Disease - Primary (2)
A

Young females with exposure to extreme stress and/or temperatures

Cause Unknown

68
Q
  1. Vasculitis with Other Disorders: Raynaud’s Phenomenon & Disease - Secondary Defined
A

Arterial insufficiency of digital vessels in response to cold or increased emotion

69
Q
  1. Vasculitis with Other Disorders: Raynaud’s Phenomenon & Disease - Causes of Secondary (7)
A
  1. TAO
  2. Takayasy’s arteritis
  3. Cryoglobulinemia
  4. Ergot Poisoning
  5. Thoracic Outlet Syndrome
  6. Cold agglutinin disease
  7. CREST syndrome
70
Q
  1. Vasculitis with Other Disorders: Raynaud’s Phenomenon & Disease - Clinical Features (5)
A
  1. Young woman
  2. *Episodic small artery vasospasm in extremities, nose, or ears
  3. Blanching and cyanosis of fingers or toes
  4. Precipitated by cold temperature/emotions
  5. *No under underlying disease or pathology
71
Q
  1. Vasculitis with Other Disorders: Raynaud’s Phenomenon & Disease - Feature of Fingers (3)
A

Proximally: Vasodialtion and Redness

Middle: Paleness due to vasconstriction

Tip: Cyanosis

72
Q
  1. Vasculitis with Other Disorders: Henoch-Schonlein Purpura is what type of hypersensitivity reaction?
A

Type III Hypersensitivity

73
Q
  1. Vasculitis with Other Disorders: Henoch-Schonlein Purpura - Defined (7)
A

Immune vasculitis

  • Usually occurs in children following URTI
  • Most common vasculitis in children
  • IgA-C3 immunocomplexes deposit in vessel wall
  • IgA nephropathy (Berger’s disease) may be part of the systemic complex
  • Purpuras over EXTENSOR SURFACE - arm, forearm, buttock, leg
74
Q
  1. Vasculitis with Other Disorders: Henoch-Schonlein Purpura - *Features (4)
A
  1. Palpable Purpura - lower extremities, buttocks
  2. Polyarthritis
  3. Abdominal Pain (sometimes with melena)
  4. Renal Disease (Hematuria)
75
Q
  1. Vasculitis with Other Disorders: Varicose Veins - Defined
A

Abnormally dilated, and tortuous veins

76
Q
  1. Vasculitis with Other Disorders: Varicose Veins - Sites (4)
A
  1. Superficial saphenous veins (connect to deep veins via perforated veins)
  2. Distal esophagus in portal HTN (from liver cirrhosis)
  3. Anorectal Region (Hemorrhoids)
  4. Left Testicle Vein (Acute angle and drains into left kidney, seen in renal cell carcinoma)
77
Q
  1. Vasculitis with Other Disorders: Varicose Veins - Primary (2)
A
  1. Due to valvular incompetence and weakened vessel walls (long standing jobs)
  2. Positive family or occupational history
78
Q
  1. Vasculitis with Other Disorders: Varicose Veins - Secondary
A

Due to valve damage from **previous thrombophlebitis or DVT

79
Q
  1. Vasculitis with Other Disorders: Varicose Veins - Epidemiology (2)
A

F>M

15% of US Pop

80
Q
  1. Vasculitis with Other Disorders: Varicose Veins - Predisposing Factors (4)
A
  1. *Obesity
  2. *Pregnancy
  3. Long standing jobs/airplane
  4. > 50 years
81
Q
  1. Vasculitis with Other Disorders: Varicose Veins - Complications (2)
A
  1. Stasis Dermatitis

2. Ulcerations from scratching

M3 Path Block 2 (10 decks)

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