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MHD V: Renal > 11.10: Vasculitis > Flashcards

11.10: Vasculitis Flashcards

1
Q

Types of immune mediated inflammation leading to vasculitis?

A
  1. Immune complex associated
  2. ANCA: antineutrophil cytoplasmic antibody
  3. Anti endothelial cell antibodies: direct vasc. attack
  4. Cell mediated immunity
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2
Q

Broad categories that can cause vasculitis?

A
  1. Immune mediated

2. Invasion by infectious pathogens

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3
Q

Does vasculitis occur mainly in venous or arterial system?

A

Arterial

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4
Q

Layers of vessels from inside to out?

A
  1. Intima
  2. Internal elastic lamina
  3. Media
  4. Adventitia
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5
Q

How does vasculitis move in small vessels?

A

From inside out

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6
Q

How does vasculitis move in large vessels?

A

Vaso vasorum in the adventitia can allows immune cells and pathogens to infiltrate
**Inside out in medium vessels as well

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7
Q

Is vasculitis and inside out or outside in process? Which type of cells are responsible?

A
  • Inside to outside: in small and medium vessels where most occurs
  • Normally mediated by neutrophils
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8
Q

How are neutrophils involved?

A
  • Neut is traveling in blood then adheres to vessel wall

- Something causes degranulation leading to inflammation

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9
Q

Pathway in large vessels?

A
  • Naive T Cells activate DCs in adventitia
  • Activation cause ROS = damage of media
  • Non scarring arteritis: window of opportunity?
  • Scarring and fibrosis break down elastic lamina and thicken Intima
  • Fibrosis = stenosis / ischemia
  • Breakdown of elastic lamina = aneurysm
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10
Q

Difference between instigating cells in small and large vessels?

A

Small: Neutrophils
Large: Macrophages

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11
Q

Pathway in small vessel vasculitis?

A
  • Inflammatory state is first step
  • Next several things could happen:
    a. IC attaches and irritates endothelium
    b. Anti neutrophil Ig could active neut
    c. Ig directly bind endothelium leading to neut attack
  • Neutrophils then degranulate causing inflammation
  • Small vessel comes apart leaking blood
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12
Q

What happens to endothelial cell when activated?

A
  • Procoagulant: express adhesion factors
  • Proinflammatory
  • Leads to turbulent flow
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13
Q

What are features of all vasculitides?

A
  1. Fever
  2. Fatigue
  3. Myalgia
  4. Weight loss
  5. Organ dysfunction
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14
Q

What is CRP?

A
  • Biomarker for inflammation

- Produced by liver

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15
Q

What occurs when levels of fibrinogen rise?

A
  • Sedimentation rate goes up
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16
Q

What is leukocytoclasis?

A
  • Neutrophils are exploding like kamikazes leaving nuclear debris in wake
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17
Q

Characteristics of small vessel vasculitis?

A
  1. Leukocytoclasis
  2. Fibrinoid necrosis
  3. Possible Immune complex deposition
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18
Q

What is fibrinoid necrosis?

A
  • Dead neutrophils with fibrin deposition
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19
Q

Clinical signs of small vessel vasculitis?

A
  1. Palpable purpura: bumpy and does not blanch
  2. Bleeding in small vessels of lungs
  3. Necrotizing glomerulonephritis
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20
Q

What is special about purpura in vasculitis?

A
  • It is bumpy from blood vessel inflammation causing them to pop up
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21
Q

Features of medium vessel vasculitis?

A
  • Muscular layer offers great protection from obliteration
    1. Fibrinoid necrosis with pleomorphic infiltrate
    2. Fibrinoid necrosis is focal and segmental: not entire circumference, skip along vessel
    3. Aneurysms from destruction of media
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22
Q

Clinical findings in medium cell vasculitis?

A
  1. Skin nodules in hands
  2. Livedo: Ringlike rash
  3. Ulcers instead of palpable purpura
  4. Abdominal pain
  5. Nerve infarcts: sensory and motor loss
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23
Q

What causes large vessel vasculitis?

A
  • Usually not neutrophilic like small and medium
  • Can be immune complex mediated
  • Usually innate cells such as macs and T cells
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24
Q

Characteristics of large vessel vasculitis?

A
  • Innate cell mediated
  • Outside in
  • Granulomas starting in adventitia usually present
  • Intima becomes hyperplastic leading to stenosis / occlusion
  • Neovascularization occurs to get some blood through
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25
Q

When does fibrinoid necrosis occur?

A
  • Small and medium vasculitis, note large
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26
Q

Clinical findings in large vessel vasculitis?

A
  1. Loss of vision or 2x vision
  2. Claudication: cramps from low flow
  3. Chest pain
  4. Headaches and stroke symptoms
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27
Q

Diagnostic considerations in vasculitis?

A
  1. Vessel size based on presentation
  2. ANCA association
  3. Granulomas
  4. Possible disease association
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28
Q

Study performed with large vessel involvement?

A
  • Angiography or MRA
  • ESR
  • CRP
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29
Q

Study performed with small vessel involvement?

A
  • CT Chest
  • Lung / kidney biopsy
  • Skin biopsy
  • Urinalysis
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30
Q

What are two types of ANCA? How to tell which kind?

A
  1. C-ANCA, “Cytoplasmic:” Elisa test positive for PR3

2. P-ANCA “Perinuclear:” Elisa test positive for MPO

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31
Q

What is Elisa test positive for PR3 indicative of?

A

C-ANCA

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32
Q

What is Elisa test positive for MPO indicative of?

A

P-ANCA

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33
Q

Pathogenesis of ANCA?

A
  • Infection creating proinflammatory state
  • Antibodies can be developed for neut granules
  • Antibodies will later stimulate neut w/o infection
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34
Q

What can be used to treat ANCA?

A

Rituxan

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35
Q

Why does knowing if there are granulomas help?

A

There are small amount of vasculitis that form them

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36
Q

What are some non microbial antigens that can cause vasculitis?

A
  1. Drugs
  2. Allergens
  3. Tumor antigens
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37
Q

What can vasculitis be secondary to?

A
  1. HIV
  2. Lupus
    3 Rheumatoid arthritis
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38
Q

What is GCA?

A

“Giant Cell Arteritis”

  • > 50 yo, w/ 2/3 females
  • More common in scandinavians: whites
  • Less common in blacks, hispanics, asians
  • Large / medium vessels
  • Tender and tortuous temporal artery
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39
Q

Where does GCA usually occur?

A
  • Usually in external carotid and temporal: tender and tortuous
  • Can occur in ophthalmic c. off internal carotid
  • Does NOT involve cerebral arteries
  • Aorta
40
Q

Pathologic features of GCA?

A
  1. Patchy and segmental
  2. Granulomatous
  3. Fragmented IEL: Aneurysm
  4. Can be chronic
41
Q

Clinical features of GCA?

A
  1. Headaches
  2. Polymyalgia Rheumatic: stiff in morning
  3. Scalp tenderness
  4. TA pulse abnormality
  5. Ocular disturbance
  6. Claudication in extremities
  7. Cardiac or neurologic symptoms
42
Q

Investigations for GCA?

A
  1. Temporal artery biopsy
  2. Test acute phase proteins
  3. Vessel imaging for claudication
43
Q

Treatment for GCA?

A

Corticosteroids with SLOW taper

44
Q

Another name for pulseless disease?

A

Takayasu’s ateritis

45
Q

Typical ptn. with pulseless disease?

A

Japanese female

46
Q

Vessel normally involved in Takayasu’s arteritis?

A
  • Aorta, arch, and branches
  • Pulmonary artery
  • Renal artery
  • Cerebral arteries with stroke
  • **Likes to stenose in branching areas
47
Q

Pathologic findings in Takayasu’s?

A
  • Very similar to GCA
  • Intense transmural scarring
  • Hyperplastic intima with neovascularization
  • Luminal narrowing
48
Q

3 phases of Takayasu’s?

A
  1. Inflammatory period: ever, pain etc
  2. Vessel inflammation with pain and tenderness
  3. Fibrotic state with stenosis and bruits
49
Q

Clinical features of Takayasu’s?

A
  1. Abnormal pulses
  2. Unequal blood pressure
  3. Bruits
  4. Claudication
  5. Ocular disturbance
  6. Pulmonary / renal htn
50
Q

Key diagnostic test in Takayasu’s?

A
  • Imaging of aorta and all vessels
51
Q

What is most common cause of heart disease in kids?

A

Kawasaki’s

52
Q

Pathologic characteristics of Kawasaki’s?

A
  1. Fibrinoid necrosis
  2. IEL destruction
  3. NO granulomas
  4. Aneurysm or acute thrombosis
53
Q

When does kawasaki’s need to be treated?

A

2 weeks after first fever

54
Q

Clinical features of Kawasaki’s disease?

A
  1. Fever
  2. Polymorphous rash
  3. Bilateral conjunctival w/o exudate
  4. Strawberry mouth and tongue
  5. Edema and erythema in extremities
  6. Swollen glands
  7. Coronary aneurysm
55
Q

Treatment of Kawasaki’s disease?

A
  • High dose aspirin

- IVIG: within first ten days of fever

56
Q

What is PAN?

A

“Polyarteritis Nodosa”

  • Small and medium muscular arteries
  • Can be caused by hep B / C
57
Q

What arteries does PAN prefer?

A
  1. Renal
  2. Coronary
  3. Hepatic
58
Q

Pathologic features of PAN?

A
  • Focal
  • Segmental
  • Transmural
  • Fibrinoid necrosis
59
Q

Clinical features of PAN?

A
  • Organ infarct
  • Abdominal pain
  • Arthralgia
  • Neuropathy: asymmetrically additive
  • LUNGS ARE SPARED
60
Q

What is characteristic of PAN neuropathy?

A

Asymmetrically additive: “Mononeuritis multiplex”

  • Won’t be entire hand or foot as in diabetes
  • Will just be individual parts
61
Q

What is Mononeuritis multiplex?

A
  • Asymmetrically additive neuropathy seen in PAN
62
Q

Is PAN ANCA positive or negative?

A

Negative

63
Q

Tests for PAN?

A
  • ANCA negative

- Angiogram with smooth stenosis followed by aneurysm

64
Q

How to treat PAN?

A
  • Corticosteroids

- Plasma exchange if from Hep B

65
Q

What are the 3 ANCA associated vasculitis?

A
  1. GPA: “Granulomatosis with Polyangiitis”
  2. MPA: “Microscopic polyangiitis”
  3. CS: Churg Strauss
66
Q

Symptoms of ANCAs?

A
  • Palpable purpura
  • Renal / pulmonary syndrome
  • Impact small vessels
67
Q

What size vessels do ANCAs impact?

A
  • Small and some medium

- Kidneys / lungs

68
Q

Pathologic features of ANCAs?

A
  • **All are Pauci immune
    1. GPA:
  • Leukocytoclastic fibrinoid necrosis
  • Granulomas in upper resp tract
    2. MPA: “Microscopic polyangiitis
  • Leukocytoclastic fibrinoid necrosis
  • NO granulomas
    3. CS: Churg Strauss
  • Leukocytoclastic fibrinoid necrosis
  • Granulomas
  • Peripheral and tissue eosinophelia
69
Q

Which ANCA shows no granuloma?

A

MPA

70
Q

Which ANCA shows peripheral and tissue eosinophilia?

A

CS

71
Q

What does pauci immune mean?

A
  • Not much immune deposition

- Immunofluorescence is not positive

72
Q

Clinical features of GPA?

A
  • Destruction of upper airway: SADDLE NOSE
  • Gingival hyperplasia
  • Subepiglottic stenosis
  • Eye issues
  • Necrotic pulmonary cavities
  • NCGN: Necrotizing crescentic glomerulonephritis
73
Q

Clinical features of MPA?

A
  • Alveolar

- NCGN

74
Q

Clinical features of CS?

A
  • Nasal polyps
  • Asthma / allergy
  • NCGN
  • Can progress to cardiomyopathy from eosinophilic cardia infiltrate
75
Q

Test indicating GPA?

A
  • C ANCA and PR3 positive
76
Q

What is C ANCA and PR3 positive indicative of?

A

GPA

77
Q

Test indicating MPA?

A
  • P ANCA and MPO positive
78
Q

What does P ANCA and MPO positive indicate?

A

MPA

- Lesser likelihood of CS

79
Q

What will ANCAs show on urine studies?

A
  • Proteinuria
  • RBCs
  • Casts
80
Q

What test is expected positive in Immune complex mediated vasculitis?

A
  • Immunofluorescence
81
Q

What is HSP?

A

“Henoch Schonlein Purpura”

  • Usually in children: adults too
  • More common in winter
  • 50% of time preceded by URI
  • Small vessels: IC mediated
82
Q

What are the immune complex mediated diseases?

A
  1. SLE vasculitis
  2. HSP
  3. Cryoglobulin vasculitis
  4. Goodpasture disease
    * *All occur in small vessels
83
Q

What is IgA and C3 on immunofluorescence characteristic of?

A

HSP

84
Q

How do you test for HSP?

A
  1. IgA and C3 on neutrophils

2. H and E shows neutrophils

85
Q

HSP clinical features?

A
  1. Palpable purpura
  2. Arthritis
  3. Abdominal pain
  4. Renal impairment
86
Q

What are the following characteristic of?

  1. Palpable purpura
  2. Arthritis
  3. Abdominal pain
  4. Renal impairment
A

HSP

87
Q

HSP treatment?

A
  • Hydration

- NSAIDs if no renal involvement

88
Q

What is thromboangiitis obliterans?

A
  • Seen in smokers
89
Q

What are corkscrew collaterals indicative of?

A

Thromboangiitis obliterans

90
Q

Treatment for thromboangiitis obliterans?

A

STOP SMOKING YOU IDIOT

91
Q

Vasculitis treatment?

A
  1. Steroids
  2. Chemo
  3. Rituximab in B cell
92
Q

When would you see granulomas at IEL?

A

GCA

93
Q

When do you see leukocytoclastic vasculitis in glomerular capillaries?

A

ANCA associated / small vessel

94
Q

When do you see extensive hyperplastic intima with neovascularization?

A

Takayasu’s

95
Q

When do you see focal vasculitis with transmural fibrinoid necrosis?

A

PAN

96
Q

What disease can cause aortic dissection?

A

GCA

MHD V: Renal (28 decks)

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