11.10: Vasculitis Flashcards Preview

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Flashcards in 11.10: Vasculitis Deck (96):
1

Types of immune mediated inflammation leading to vasculitis?

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

2

Broad categories that can cause vasculitis?

1. Immune mediated
2. Invasion by infectious pathogens

3

Does vasculitis occur mainly in venous or arterial system?

Arterial

4

Layers of vessels from inside to out?

1. Intima
2. Internal elastic lamina
3. Media
4. Adventitia

5

How does vasculitis move in small vessels?

From inside out

6

How does vasculitis move in large vessels?

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

7

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

- Inside to outside: in small and medium vessels where most occurs
- Normally mediated by neutrophils

8

How are neutrophils involved?

- Neut is traveling in blood then adheres to vessel wall
- Something causes degranulation leading to inflammation

9

Pathway in large vessels?

- 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

10

Difference between instigating cells in small and large vessels?

Small: Neutrophils
Large: Macrophages

11

Pathway in small vessel vasculitis?

- 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

12

What happens to endothelial cell when activated?

- Procoagulant: express adhesion factors
- Proinflammatory
- Leads to turbulent flow

13

What are features of all vasculitides?

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

14

What is CRP?

- Biomarker for inflammation
- Produced by liver

15

What occurs when levels of fibrinogen rise?

- Sedimentation rate goes up

16

What is leukocytoclasis?

- Neutrophils are exploding like kamikazes leaving nuclear debris in wake

17

Characteristics of small vessel vasculitis?

1. Leukocytoclasis
2. Fibrinoid necrosis
3. Possible Immune complex deposition

18

What is fibrinoid necrosis?

- Dead neutrophils with fibrin deposition

19

Clinical signs of small vessel vasculitis?

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

20

What is special about purpura in vasculitis?

- It is bumpy from blood vessel inflammation causing them to pop up

21

Features of medium vessel vasculitis?

*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

22

Clinical findings in medium cell vasculitis?

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

23

What causes large vessel vasculitis?

- Usually not neutrophilic like small and medium
- Can be immune complex mediated
- Usually innate cells such as macs and T cells

24

Characteristics of large vessel vasculitis?

- 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

25

When does fibrinoid necrosis occur?

- Small and medium vasculitis, note large

26

Clinical findings in large vessel vasculitis?

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

27

Diagnostic considerations in vasculitis?

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

28

Study performed with large vessel involvement?

- Angiography or MRA
- ESR
- CRP

29

Study performed with small vessel involvement?

- CT Chest
- Lung / kidney biopsy
- Skin biopsy
- Urinalysis

30

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

1. C-ANCA, "Cytoplasmic:" Elisa test positive for PR3
2. P-ANCA "Perinuclear:" Elisa test positive for MPO

31

What is Elisa test positive for PR3 indicative of?

C-ANCA

32

What is Elisa test positive for MPO indicative of?

P-ANCA

33

Pathogenesis of ANCA?

- Infection creating proinflammatory state
- Antibodies can be developed for neut granules
- Antibodies will later stimulate neut w/o infection

34

What can be used to treat ANCA?

Rituxan

35

Why does knowing if there are granulomas help?

There are small amount of vasculitis that form them

36

What are some non microbial antigens that can cause vasculitis?

1. Drugs
2. Allergens
3. Tumor antigens

37

What can vasculitis be secondary to?

1. HIV
2. Lupus
3 Rheumatoid arthritis

38

What is GCA?

"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

39

Where does GCA usually occur?

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

40

Pathologic features of GCA?

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

41

Clinical features of GCA?

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

Investigations for GCA?

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

43

Treatment for GCA?

Corticosteroids with SLOW taper

44

Another name for pulseless disease?

Takayasu's ateritis

45

Typical ptn. with pulseless disease?

Japanese female

46

Vessel normally involved in Takayasu's arteritis?

- Aorta, arch, and branches
- Pulmonary artery
- Renal artery
- Cerebral arteries with stroke
***Likes to stenose in branching areas

47

Pathologic findings in Takayasu's?

*Very similar to GCA
- Intense transmural scarring
- Hyperplastic intima with neovascularization
- Luminal narrowing

48

3 phases of Takayasu's?

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

49

Clinical features of Takayasu's?

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

50

Key diagnostic test in Takayasu's?

- Imaging of aorta and all vessels

51

What is most common cause of heart disease in kids?

Kawasaki's

52

Pathologic characteristics of Kawasaki's?

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

53

When does kawasaki's need to be treated?

2 weeks after first fever

54

Clinical features of Kawasaki's disease?

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

Treatment of Kawasaki's disease?

- High dose aspirin
- IVIG: within first ten days of fever

56

What is PAN?

"Polyarteritis Nodosa"
- Small and medium muscular arteries
- Can be caused by hep B / C

57

What arteries does PAN prefer?

1. Renal
2. Coronary
3. Hepatic

58

Pathologic features of PAN?

- Focal
- Segmental
- Transmural
- Fibrinoid necrosis

59

Clinical features of PAN?

- Organ infarct
- Abdominal pain
- Arthralgia
- Neuropathy: asymmetrically additive
- LUNGS ARE SPARED

60

What is characteristic of PAN neuropathy?

Asymmetrically additive: "Mononeuritis multiplex"
- Won't be entire hand or foot as in diabetes
- Will just be individual parts

61

What is Mononeuritis multiplex?

- Asymmetrically additive neuropathy seen in PAN

62

Is PAN ANCA positive or negative?

Negative

63

Tests for PAN?

- ANCA negative
- Angiogram with smooth stenosis followed by aneurysm

64

How to treat PAN?

- Corticosteroids
- Plasma exchange if from Hep B

65

What are the 3 ANCA associated vasculitis?

1. GPA: "Granulomatosis with Polyangiitis"
2. MPA: "Microscopic polyangiitis"
3. CS: Churg Strauss

66

Symptoms of ANCAs?

- Palpable purpura
- Renal / pulmonary syndrome
- Impact small vessels

67

What size vessels do ANCAs impact?

- Small and some medium
- Kidneys / lungs

68

Pathologic features of ANCAs?

***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

Which ANCA shows no granuloma?

MPA

70

Which ANCA shows peripheral and tissue eosinophilia?

CS

71

What does pauci immune mean?

- Not much immune deposition
- Immunofluorescence is not positive

72

Clinical features of GPA?

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

73

Clinical features of MPA?

- Alveolar
- NCGN

74

Clinical features of CS?

- Nasal polyps
- Asthma / allergy
- NCGN
- Can progress to cardiomyopathy from eosinophilic cardia infiltrate

75

Test indicating GPA?

- C ANCA and PR3 positive

76

What is C ANCA and PR3 positive indicative of?

GPA

77

Test indicating MPA?

- P ANCA and MPO positive

78

What does P ANCA and MPO positive indicate?

MPA
- Lesser likelihood of CS

79

What will ANCAs show on urine studies?

- Proteinuria
- RBCs
- Casts

80

What test is expected positive in Immune complex mediated vasculitis?

- Immunofluorescence

81

What is HSP?

"Henoch Schonlein Purpura"
- Usually in children: adults too
- More common in winter
- 50% of time preceded by URI
- Small vessels: IC mediated

82

What are the immune complex mediated diseases?

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

83

What is IgA and C3 on immunofluorescence characteristic of?

HSP

84

How do you test for HSP?

1. IgA and C3 on neutrophils
2. H and E shows neutrophils

85

HSP clinical features?

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

86

What are the following characteristic of?
1. Palpable purpura
2. Arthritis
3. Abdominal pain
4. Renal impairment

HSP

87

HSP treatment?

- Hydration
- NSAIDs if no renal involvement

88

What is thromboangiitis obliterans?

- Seen in smokers

89

What are corkscrew collaterals indicative of?

Thromboangiitis obliterans

90

Treatment for thromboangiitis obliterans?

STOP SMOKING YOU IDIOT

91

Vasculitis treatment?

1. Steroids
2. Chemo
3. Rituximab in B cell

92

When would you see granulomas at IEL?

GCA

93

When do you see leukocytoclastic vasculitis in glomerular capillaries?

ANCA associated / small vessel

94

When do you see extensive hyperplastic intima with neovascularization?

Takayasu's

95

When do you see focal vasculitis with transmural fibrinoid necrosis?

PAN

96

What disease can cause aortic dissection?

GCA