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Y2 MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is polymyalgia rheumatic

A

Common chronic inflammatory condition of unknown cause that affects mainly elderly individuals

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2
Q

Polymyalgia rheumatica mainly affects which age group

A

those over 50

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3
Q

Polymyagia rheumatic is associated with which condition

A

Giant cell arteritis
-40% with GCA develop PR
-15% PR develop GCA

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4
Q

Risk factors for polymyalgia rheumatica

A

Over 50
Northern European descent

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5
Q

Which joints are commonly affected by polymyalgia rheumatica

A

Shoulder
Hips

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6
Q

Symptoms of polymyagia rheumatica

A

Symmetrical myalgia of hip and shoulder girdle
Stiffness > 45m
Pain worse with movement
Low fever
Reduced appetite
Weight loss
Malaise

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7
Q

What can relief the pain caused by polymyalgia rheumatica

A

Oral steroids

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8
Q

Clinical signs of polymyalgia rheumatic

A

Limited joint movement due to pain
Normal muscle strength
Tenderness

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9
Q

Dermatomyositis also causes myalgia. What are the differences between polymyalgia rheumatica and dermatomyositis

A

PR does not cause muscle weakness whereas dermatomyositis does
PR causes pain whereas dermatomyositis doesn’t really cause pain

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10
Q

investigations for polymyagia rheumatica

A

Clinical
Bloods - ESR and CRP (raised)
therapeutic trial of steroids

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11
Q

Management of polymyalgia rheumatica

A

investigations before starting steroids
Oral steroid therapy- prednisolone

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12
Q

Describe the steroid therapy for polymyalgia rheumatica

A

Does not need to be started right away - do check ups before starting
Start prednisolone at 15
Gradually decrease the dose
Symptoms should resolve

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13
Q

What investigations need to be done before starting steroids

A

Screen for
- diabetes
- peptic ulcers
- osteoporosis (DEXA scan)
- mental health history
- hypertension

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14
Q

2 types of large vessel vasculitis

A

Takayasu’s arteritis
Giant cell arteritis

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15
Q

Which vessels are affected by Takayasu’s arteritis

A

Aorta
Main branches of the aorta

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16
Q

Risk factors for takayasu’s arteritis

A

Female
<40 years old
Asian

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17
Q

What can vascular inflammation lead to

A

stenosis
occlusion
aneurysm

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18
Q

Symptoms of takayasu’s arteritis

A

Systemic upset - mild fever / malaise / weight loss
Arthralgia
Night sweats
Claudication in upper and lower limbs
Skin nodules

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19
Q

Clinical signs of takayasu’s arteritis

A

Absent upper extremity pulse
Difference in blood pressure between the extremities
Bruit
Aortic regurgitaiton

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20
Q

What is bruit

A

audible vascular sound associated with turbulent blood flow

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21
Q

What causes bruit in takayasu’s arteritis

A

vascular stenosis / aneurysms

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22
Q

Investigations for takayasu’s arteritis

A

Bloods - inflammatory markers raised
MR Angiogram

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23
Q

Management for takayasu’s arteritis

A

Steroids

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24
Q

Which arteries are most commonly affected by giant cell arteritis (temporal arteritis)

A

Temporal arteries - arteries around the temples

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25
Q

Risk factors for GCA

A

> 50 years, most commonly late 60+
Polymyalgia rheumatica

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26
Q

Symptoms of GCA

A

temporal headache
Jaw claudication - pain on chewing food
Amaurosis fugax
Visual disturbances
Thickened temporal artery
Scalp tenderness
polymyagia rheumatica symptoms

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27
Q

What is amaurosis fugax

A

transient loss of vision in one or both eyes
“dark curtain descending”

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28
Q

Clinical signs of GCA

A

Non-pulsatile, thickened temporal arteries

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29
Q

Complications of GCA

A

Permanent blindness
Stroke

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30
Q

Investigations for GCA

A

Bloods - ESR
Temporal artery US
Temporal artery biopsy
CT angiogram / MR angiogram if still unsure

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31
Q

When should you suspect GCA in a patient

A

Patient over 50 years old, new onset of headache with raised inflammatory markers

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32
Q

What is important to be measured in bloods for GCA

A

ESR

33
Q

What can be seen on temporal artery biopsy in GCA

A

Transmural inflammation of the layers of arteries
Patchy infiltration by giant cells, lymphocytes
Thickened vessel wall

34
Q

Why does temporal artery biopsy have low sensitivity but high specificity for GCA

A

Low sensitivity due to skip lesions
= biopsy may be taken from a normal segment so a negative biopsy does not rule out the disease

35
Q

Management of GCA

A

Immediate Oral steroids - prednisolone
Gradually reduce steroids dosage
Steroid sparing agents if cannot reduce steroid dosage

36
Q

What are the steroid sparing agents

A

Methotrexate
Azathioprine
Leflunamide

37
Q

Why should you reduce the steroid dosage gradually instead of quickly

A

Because symptoms can relapse

38
Q

Differences between takayasu’s arteritis and GCA

A

Takayasu arteritis mainly affects the aorta whereas GCA affects temporal arteries
Takayasu arteritis mainly affects younger patients whereas GCA affects older patients

39
Q

Common biopsy finding between takayasu and GCA

A

skip lesions

40
Q

What is ANCA associated small vessel vasculitis

A

Group of autoimmune inflammation conditions of small to medium vessels associated with anti-neutrophil cytoplasmic antibodies (ANCA)

41
Q

What are the conditions part of ANCA vasculitis

A

Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis

42
Q

What is granulomatosis with polyangiitis

A

Granulomatous inflammation affecting small and medium sized vessels in the upper and lower resp tract, eyes, kidneys

43
Q

Classic triad of granulomatosis with polyangiitis

A

Upper resp tract involvement
Lower resp tract involvement
Pauci-immune glomerulonephritis

44
Q

What are the signs of URT involvement in granulomatosis with polyangiitis

A

Chronic sinusitis
Epistaxis
Saddle nose deformity
Otitis media
Mouth ulcers
Sensorineural deafness

45
Q

What are the signs of LRT involvement in granulomatosis with polyangiitis

A

Haemoptysis
Cough
Pleuritis
Alveolar haemorrhage
Bilateral cavitating nodules on CXR

46
Q

What is pauci immune glomerulonephritis

A

Rapidly progressive condition leading to renal failure within days, strongly associated with ANCA

47
Q

Presentation of pauci-immune glomerulonephritis

A

Proteinuria
Haematuria
Frothy urine due to proteinuria

48
Q

What are the ocular involvement of granulomatosis with polyangiitis

A

Conjunctivitis
Optic nerve vasculitis
Retinal artery occlusion
Uveitis
Proptosis

49
Q

What are the nervous system manifestations of GPA

A

mononeuritis multiplex
polyneuropathy

50
Q

What is mononeuritis multiplex

A

peripheral neuropathy of a single nerve at least 2 different areas of peripheral nervous system

51
Q

Difference between mono neuritis multiplex and polyneuropathy

A

Polyneuropathy is damage to multiple nerves

52
Q

What is eosinophilic granulomatosis with polyangiitis

A

granulomatous inflammation mostly affecting small and medium vessels in resp tract and skin

53
Q

Diagnostic criteria of EGPA

A

Late onset adult asthma
Eosinophilia
Paranasal sinusitis
Pulmonary infiltrates
Histological confirmation of vasculitis
Neuropathy - mononeuritis multiplex / polyneuropathy

54
Q

Difference between granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

A

EGPA has high eosinophil count
EGPA causes late onset adult asthma whereas GPA doesn’t

55
Q

What is microscopic polyangiitis

A

Small to medium vessel vasculitis characterised by renal, pulmonary disease and pANCA

56
Q

Which symptom is very common in microscopic polyangiitis

A

Necrotising glomerulonephritis

57
Q

Symptoms of microscopic polyangiitis

A

Necrotising glomerulonephritis
Palpable purpura
Neuropathy
Alveolar haemorrhage
Haemoptysis

58
Q

Investigations for ANCA- associated polyangiitis

A

Bloods
Immunofluorescence
Biopsy of affected areas

59
Q

What may blood tests show in ANCA-associated polyangiitis

A

Raised inflammatory markers
Anaemia
Low complement level during active disease

60
Q

What does immunofluorescence look at in ANCA associated polyangiitis

A

ANCA
PR3
MPO

61
Q

What are ANCA

A

autoantibodies against antigens in the cytoplasm of neutrophils

62
Q

ANCA present in GPA

A

cANCA

63
Q

ANCA present in EGPA

A

pANCA

64
Q

ANCA present in MPA

A

pANCA

65
Q

Can ANCA be diagnostic for ANCA associated polyangiitis

A

No, because a proportion of patients may be ANCA negative
and the level of those autoantibodies vary with disease level

66
Q

Management of ANCA associated polyangiitis depends on

A

involvement of organs
- localised
- early systemic
- generalised
- systemic
- unresponsive

67
Q

Localised ANCA associated polyangiitis =

A

only upper +/- lower resp tract involved

68
Q

generalised ANCA associated polyangiitis =

A

Early renal involvement

69
Q

Systemic ANCA associated polyangiitis =

A

Late renal involvement
Vital organ failure

70
Q

Management of localised and early systemic ANCA associated polyangiitis

A

Methotrexate + steroids

71
Q

Management of generalised ANCA associated polyangiitis

A

Cyclophosphamide + steroids
Azathioprine for maintenance

72
Q

Management of systemic ANCA associated polyangiitis

A

Cyclophosphamide + steroids
Plasma exchange if creatine > 500
Azathioprine for maintenance

73
Q

Management of refractory ANCA associated polyangiitis

A

IV immunoglobulins
Rituximab

74
Q

What is Henoch-Schonlein Purpura

A

IgA mediated generalised vasculitis affecting small vessels of the skin, GI, kidneys, joints

75
Q

Which age group is most commonly affected by Henoch-Schonlein Purpura

A

2-11 years old

76
Q

Patients with Henoch-Schonlein Purpura usually had which preceding infections

A

Group A strep infection
viral URTI
pharyngeal infection
GI infection

77
Q

Symptoms of Henoch-Schonlein Purpura

A

Purpuric rash over buttocks and lower limbs
Abdominal pain
Bloody diarrhea
Arthralgia
Nephritis

78
Q

Investigations for Henoch Schonlein Purpura

A

Clinical
Urine dip stick
Bloods

79
Q

Management for Henoch Schonlein Purpura

A

Self limiting
Regular urine dips for 12 months to check for renal impairment

Y2 MSK (19 decks)

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