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Vasculitis Flashcards

1
Q

VASCULITIS refers to _____ and ____ of ____

A

inflammation and necrosis

blood vessels

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2
Q

VASCULITIS only occurs in arteries

T/F

A

F

arteries, veins and capillaries

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3
Q

In VASCULITIS

These vessels may be damaged by
__________,_________,__________,_________ or _____

A

immune mechanisms, infectious agents, mechanical trauma, radiation or toxins

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4
Q

Majority of VASCULITIS are thought to involve immune mechanisms including;
- _______ of immune complexes
- ________ on vessels by _______
- various forms of _______

A

deposition

direct attack ; circulating antibodies

cell mediated immunity

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5
Q

BASIC PRINCIPLES of vasculitis
Inflammation of the blood vessel wall

• Arterial wall is comprised of three layers:_____ intima, _______ media, and _________ adventitia

•Etiology is usually (known or unknown?)

A

endothelial; smooth muscle ; connective tissue

unknown

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6
Q

BASIC PRINCIPLES of vasculitis

Clinical features include

Non specific symptoms of inflammation(e.g., ____,______,_____, and ______)

Symptoms of _______: due to _______ or ______ of the inflamed vessels

A

fever,fatigue, weight loss, and myalgias

Organ Ischemia; luminal narrowing or thrombosis

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7
Q

BASIC PRINCIPLES of vasculitis

Divided into _________,________,_______

A

large-, medium-, and small-vessel

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8
Q

BASIC PRINCIPLES of vasculitis

Large-vessel vasculitis involves the ____ and _________

Medium-vessel vasculitis involves _______ that supply organs.

Small-vessel vasculitis involves _____,______,________

A

aorta and its major branches.

muscular arteries

arterioles, capillaries, and venules.

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9
Q

most cases of vasculitis are infectious.

T/F

A

F

are not infectious

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10
Q

Mention 2 examples of large vessel vasculitis

A

Takayasu arteritis

Giant cell arteritis

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11
Q

Mention 2 examples of Medium-vessel vasculitis

A

Polyarteritis nodosa

Kawasaki disease

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12
Q

Mention 3 examples of Small-vessel vasculitis

A

Granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis

Microscopic polyangiitis

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13
Q

Some vasculitides are associated with ________________(ANCA)

A

antineutrophil cytoplasmic antibodies

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14
Q

Some vasculitides are associated with antineutrophil cytoplasmic antibodies (ANCA) :

These can be detected by ______ assays using the patient’s ______ and __________

A

indirect immunoflourescence

serum and ethanol fixed neutrophils

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15
Q

C-ANCA-

P-ANCA-

A

Cytoplasmic antineutrophil cytoplasmic antibodies

Perinuclear antineutrophil cytoplasmic antibodies

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16
Q

C-ANCA- associated with _____ antibody

P-ANCA-associated with _____ antibody

A

Proteinase-3- antibody

Myeloperoxidase antibody

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17
Q

C-ANCA-

Majorly in _______ disease (__%)

Rarely in _____,____, and ______ disease

A

Granulomatosis with Polyangitis; 90

Microscopic polyangiitis; Eosinophilic granulomatosis with polyangiitis; infective endocarditis

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18
Q

P-ANCA-

Majorly in _______ disease (__%), and ________(___%)

Rarely in _____, and ______ disease

A

Microscopic polyangiitis; 70

Eosinophilic granulomatosis with polyangiitis; 50

Granulomatosis with Polyangitis; PAN

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19
Q

Atypical P-ANCA Is found in ____ and _____

Rarely in _____ and _____

A

PSC ; ulcerative colitis

Crohn’s disease, primary biliary cirrhosis

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20
Q

GIANT CELL ARTERITIS (aka. __________,__________)

A

TEMPORAL ARTERITIS

GRANULOMATOUS ARTERITIS

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21
Q

The most common vasculitis in older adults is ???

A

GIANT CELL ARTERITIS

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22
Q

GIANT CELL ARTERITIS

Average age at onset is ___ years (___-___)

It involves a (focal or widespread ?) , (acute or chronic?) ,granulomatous inflammation

A

70; 50-75

Focal; chronic

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23
Q

GIANT CELL ARTERITIS

Usually of the _______ (small or large ?) arteries and there branches

Male : Female =_:__

A

temporal

Large

1:3

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24
Q

GIANT CELL ARTERITIS

Clinical features
Patients presents with _______ and _________

_____ symptoms occur in almost half of the patients

A

headache and throbbing temporal pain

Visual

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25
Q

GIANT CELL ARTERITIS

Clinical features

______ and _______ are present in the skin overlying the affected artery

About half with clinical manifestation will have ______ involvement

A

Swelling and tenderness

systemic

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26
Q

Lesions of temporal arteritis are focal and may be missed on biopsy

T/F

A

T

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27
Q

GIANT CELL ARTERITIS

Biopsy of the temporal artery may be negative in ____% of patients with classical manifestation of this disease

A

40

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28
Q

GRANULOMATOUS ARTERITIS
Treatment

These patients are treated with ____ and the response is (disappointing or excellent?)

A

steroids

Excellent

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29
Q

TAKAYASU ARTERITIS (_____ syndrome)

Aetiology: _______ basis has been proposed

Refers to an inflammatory disorder of the ______ and ________

A

Aortic arch

An autoimmune

aortic arch and its major branches

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30
Q

TAKAYASU ARTERITIS

Affects (young or old?) (men or women?) (<__years), 15-25

Male : Female= __:__

A

Young

Women

30

1:9

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31
Q

TAKAYASU ARTERITIS

Pathology
Aorta is _____ and has _______

A

thickened; focal raised plaques

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32
Q

Aortic arch syndrome

Pathology

Branches of the aorta display ______ and _____, interfering with _____ and accounting for the synonym ‘ ______’

A

stenosis and occlusion

blood flow

pulseless disease

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33
Q

TAKAYASU ARTERITIS

Clinical features

General: Fever, malaise, arthralgia, night sweats

Specific: Vascular symptoms
Decreased bilateral _____ and _____ (so- called pulseless disease)

_____,______
Bilateral carotid bruits
Impaired _____
_____-induced muscular pain in one or more limbs
_______ phenomenon
Hypertension

A

brachial and radial pulses

Syncope, angina pectoris

vision; Movement; Raynaud

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34
Q

POLYARTERITIS NODOSA (PAN)
A multisystemic disease
This acute, necrotizing, vasculitis affects mainly __________-sized muscular arteries

A

small to medium

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35
Q

POLYARTERITIS NODOSA (PAN)

Male:Female =___:__
40-60 years

A

2:1

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36
Q

POLYARTERITIS NODOSA (PAN)

__-ANCA (15 – 30% of cases) immunoflourescence mainly against _________

A

P

myeloperoxidase

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37
Q

Pathology of PAN

every vessel of appropriate size would be affected

A

F
Not every vessel of appropriate size would be affected so a thorough search of the slide would be necessary

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38
Q

Pathology of PAN

Lesions are _____ and may not involve the ________ of the vessel

A

patchy

entire circumference

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39
Q

Pathology of PAN

Most prominent feature is an area of the __________, fused into ________

A

media and adventitia

an eosinophilic mass

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40
Q

Inflammatory response in PAN involves only some of the adventitia

A

F

the entire adventitia (periarteritis)

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41
Q

In PAN

Healed lesions have a _____ media & loss of fragmentation of the _________

A

fibrotic

internal elastic lamina

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42
Q

internal elastic lamina is ??

A

What separates the intima from the media

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43
Q

Clinical features of PAN

specific symptoms

____ involvement
________ involvement
____ involvement
______ involvement
___ involvement

A

Renal

Coronaryartery

Skin

Neurological

GI

44
Q

Clinical features of PAN

Renal involvement (∼___%): _______ , renal impairment

Coronary artery involvement(∼__%) : increased risk of ________

Skininvolvement(∼___%): ____,_______, nodules

A

60; hypertension

35; myocardial infarction

40; rash,ulcerations

45
Q

Clinical features of PAN

Neurological involvement: polyneuropathy ( __________ ), ________

GI involvement: ______,_______, nausea, vomiting

A

mononeuritis multiplex; stroke

abdominal pain, melena

46
Q

PAN Usually spares the _____

A

lungs

47
Q

KAWASAKI DISEASE (______ SYNDROME)

A

MUCOCUTANEOUS LYMPH NODE

48
Q

KAWASAKI DISEASE

(acute or chronic?) necrotizing vasculitis of _________ and _________

A

Acute; infancy; early childhood

49
Q

KAWASAKI DISEASE

i) _____
ii) ____
iii) conjunctival and ____ lesions (characteristic “___________”
iv) lymphadenitis

A

Fever; rash ; oral

Strawberry tongue

50
Q

Kawasaki disease

Male : Female = __:__

A

1:5

51
Q

Strawberry tongue – {also seen in _______ besides Kawasaki

A

scarlet fever

52
Q

Clinical features of Giant cell Artertis

• Hardened and tender temporal artery

Jaw _______
_____ loss

Scintillating ______
________ or permanent loss of vision
Diplopia

A

claudication

Vision

scotoma

Amaurosis fugax

53
Q

Clinical features of Giant cell Artertis

• Hardened and tender temporal artery

Jaw claudication:______ when ______

Vision loss: due to _____ and ______ of the _______ and its branches

Amaurosis fugax or __________

Diplopia

A

jaw pain when chewing

inflammation and occlusion

ophthalmic artery

permanent loss of vision

54
Q

In PAN, Small aneurysm may form

T/F

A

T

55
Q

Treatment of PAN

________

_______ therapy against ______ and ____ may be required.

A

Immunosuppression

Antiviral

HBV and HCV

56
Q

KAWASAKI DISEASE

Affects ____________ sized vessels

A

large to medium

57
Q

Kawasaki disease

May affect the ____ arteries and cause _____ in ____%

_______ cause is suspected

A

coronary

aneurysms

70

An infectious

58
Q

Kawasaki Disease is not usually self-limited

T/F

A

F

Disease is usually self-limited

59
Q

THROMBOANGIITIS OBLITERANS

Occlusive inflammatory disease of _________ arteries in the _______ and _____

A

medium and small

distal arms and legs

60
Q

BUERGER DISEASE

Exacerbated by ______ which plays an aetiological role in the disease

A

smoking

61
Q

THROMBOANGIITIS OBLITERANS

An increased prevalence of ____ and ____ haplotypes among patients with the disease lends credence to the idea that a genetic _________ is involved in the pathogenesis.

A

HLA-A9 and HLA-B5

Hypersensitivity to tobacco

62
Q

THROMBOANGIITIS OBLITERANS
Aka _________ DISEASE

A

BUERGER

63
Q

BUERGER DISEASE
Pathology

Acute inflammation of the medium and small arteries, with involvement of the endothelium
May lead to ______ and ____ of the lumen

A

thrombosis and obliteration

64
Q

THROMBOANGIITIS OBLITERANS

Symptoms usually begins between ___-___ years

_______ in muscle during exercise

A

25- 40

Cramping pains

65
Q

BUERGER DISEASE

More in male or female?

Male : Female = __:__

A

Female

1:9

66
Q

BUERGER DISEASE

Painful ischaemic disease can lead to ____ of the extremity with possible necessity of ____

A

gangrene

amputation

67
Q

HYPERSENSITIVITY ANGIITIS

This is a broad category of inflammatory vascular lesions representing a reaction to foreign materials such as ______ or _____

A

bacterial products or drugs.

68
Q

HYPERSENSITIVITY ANGIITIS

It includes;
A)___________
B)_____________

A

Cutaneous vasculitis

Systemic hypersensitivity angiitis

69
Q

HYPERSENSITIVITY ANGIITIS
A) Cutaneous vasculitis
i) May follow administration of drugs such as ____,______, and ____
ii) _____ or _____ infections

A

aspirin, penicillin and thiazide diuretics

Bacterial or viral

70
Q

HYPERSENSITIVITY ANGIITIS
A) In Cutaneous vasculitis

Palpable _____ of the lower extremities is typical

Superficial cutaneous _____ display _______ and an acute inflammatory reaction

The disease is usually ______

A

purpura

venules; fibrinoid necrosis

self limited

71
Q

HYPERSENSITIVITY ANGIITIS
Systemic hypersensitivity angiitis

Also called __________

May also exhibit ______

A

microscopic polyarteritis

skin lesions

72
Q

microscopic polyarteritis

Is always a feature of other vascular diseases

A

F

May be an isolated entity or a feature of other vascular
diseases

73
Q

microscopic polyarteritis

A feared complication is _____ involvement, characterized by rapidly progressive _______ and ______

A

renal

glomerulonephritis and renal failure

74
Q

microscopic polyarteritis

It is associated with the presence of cANCA

T/F

A

T

But
Majorly pANCA

75
Q

EOSINOPHILIC/ALLERGIC
GRANULOMATOSIS WITH
POLYANGIITIS

Also known as ________ SYNDROME

A

CHURG-STRAUSS

76
Q

CHURG-STRAUSS SYNDROME occurs in young people with ____ and features _____

A

asthma; eosinophilia

77
Q

EOSINOPHILIC/ALLERGIC
GRANULOMATOSIS WITH
POLYANGIITIS

More in male or female?)

Male:Female = __:__

__-__years

A

Male

2:1

40-60

78
Q

CHURG-STRAUSS SYNDROME

Affects ___-___ sized vessels

____% exhibit C-ANCA or P-ANCA

A

small to medium

70

79
Q

CHURG-STRAUSS SYNDROME

There is widespread necrotizing vascular lesions of ________ sized arteries in multiple organs

A

small and medium

80
Q

ALLERGIC GRANULOMATOSIS AND ANGIITIS

There is prominent involvement of the _____

Other organs involved are the ____,_____,_____, and _____

A

lungs

kidneys, heart, liver and CNS

81
Q

CHURG-STRAUSS SYNDROME

Clinical features: ___________ attacks (chief complaint), Allergic _________, Skin ____, palpable _____

Gastrointestinal involvement: bleeding, ischemia, perforation

A

Severe allergic asthma

rhinitis/sinusitis

nodules; purpura

82
Q

WEGENER GRANULOMATOSIS
aka. “__________________ ”

A (Local or Systemic?) necrotizing vasculitis

A

Granulomatosis with polyangiitis

Systemic

83
Q

WEGENER GRANULOMATOSIS

Characterized by lesions of the ____,______ ,_____, and ___________ disease

Aetiology is (known or unknown?)

A

nose, sinuses, lungs and renal glomerular

unknown

84
Q

WEGENER GRANULOMATOSIS

Happens in who more?…men or women?

Male:Female = __:___

A

Both equally

1:1

85
Q

Granulomatosis with polyangiitis

Affects ____ arteries

> ___% have ANCA in their blood; of those ___% are ___-ANCA, which suggests that _________ are responsible for the attack

A

small

90; 75

C; activated neutrophils

86
Q

WEGENER GRANULOMATOSIS

Pathology
Features _______ necrosis and granulomatous inflammation composed of neutrophils and other leukocytes

Mostly in the _____,_____, and ______

A

parenchymal

respiratory tract, kidney
and spleen

87
Q

WEGENER GRANULOMATOSIS

Immunosuppressive treatment with __________ leads to striking improvement

A

cyclophosphamide

88
Q

WEGENER GRANULOMATOSIS

Clinical Features
Persistent bilateral ______ and chronic ____ are prominent

_______ and ______ indicate glomerular involvement

A

pneumonitis; sinusitis

Haematuria and proteinuria

89
Q

RAYNAUD’S DISEASE

Refers to _____ and ______ of the digits of the hands, feet, tip of the nose and ears

A

paroxysmal pallor and cyanosis

90
Q

RAYNAUD’S DISEASE

It is due to intense ________ in (old or young?) healthy (men or women?)

It is often accompanied by ____ and _____

A

local vasospasm

Young ; women

paraesthesia and pain

91
Q

RAYNAUD’S DISEASE

Usually ___ organic change in vessel wall except later on when there is _____

Usually bilateral and symmetrical and may lead to ______ and ____ of the _____

A

No; intimal proliferation

ulcers and gangrene of the tips of the digits

92
Q

RAYNAUD’S PHENOMENON

Refers to arterial _____ secondary to arterial ______

A

insufficiency

narrowing

93
Q

BEHCET DISEASE

(Focal or Widespread?) vasculitis

A

Widespread vasculitis of many organs

94
Q

BEHCET DISEASE

Characterized by _____ and ______ ; ____ inflammation; occasional lesions in the CNS, GIT & CVS

___________ vessels are involved

A

oral and genital ulcers

ocular

Large and small

95
Q

BEHCET DISEASE

20 - 35 years

Who gets it more?..men or women
Male:Female = ___:__

A

Both equally

1:1

96
Q

INFECTIOUS ARTERITIS

Caused by ______

_____ or _______ commonly

In bacterial/TB ________, bacterial ______ or pulmonary TB

A

direct invasion

Aspergillosis or mucormycosis

meningitis; pneumonia

97
Q

INFECTIOUS ARTERITIS

It is important because it induces _____ leading to _____, worsening the condition

A

thrombosis

infarct

98
Q

RAYNAUD’S PHENOMENON
Refers to arterial insufficiency secondary to arterial narrowing induced by various conditions such as
i) ___
ii) _____
iii) ________

A

SLE

SCLERODERMA

BUEGER’S DX

99
Q

More in male or female?

GIANT CELL ARTERITIS - _____

Aortic arch syndrome- _______

PAN- ______

A

female more

female only

male more

100
Q

More in male or female?

Kawasaki- _________

BUERGER DISEASE- _______

CHURG-STRAUSS SYNDROME- _______

A

female more

female more

male more

101
Q

More in male or female?

BEHCET DISEASE- ____

RAYNAUD’S DISEASE- ______

WEGENER GRANULOMATOSIS- _____

A

equal

women only

equal

102
Q

Which vasculitis have the following gender distribution

Equal - _____________________

Men- ____________________

A

wegner’s and bechet

churg Strauss and PAN

103
Q

Name and nickname!

Takayasu

Kawasaki

BUEGER’S disease

Churg Strauss

Wegner’s

Microscopic polyangitis

A

Pulseless disease/ aortic arch syndrome

Mucocutaneous lymph node syndrome

Thromboangiitis obliterans

Eosinophilic granulomatosis with polyangitis

granulomatosis with polyangitis

Systemic hypersensitivity angiitis

104
Q

Names and nicknames!!!

Pulseless disease/ aortic arch syndrome

Mucocutaneous lymph node syndrome

Thromboangiitis obliterans

Eosinophilic granulomatosis with polyangitis

granulomatosis with polyangitis

Systemic hypersensitivity angiitis

A

Takayasu

Kawasaki

BUEGER’S disease

Churg Strauss

Wegner’s

Microscopic polyangitis

105
Q

________ vaaculotis mainly causes aneurysm

_______can too

A

Kawasaksi

PAN

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