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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
Risk factors for GCA
> 50 years, most commonly late 60+ Polymyalgia rheumatica
26
Symptoms of GCA
temporal headache Jaw claudication - pain on chewing food Amaurosis fugax Visual disturbances Thickened temporal artery Scalp tenderness polymyagia rheumatica symptoms
27
What is amaurosis fugax
transient loss of vision in one or both eyes "dark curtain descending"
28
Clinical signs of GCA
Non-pulsatile, thickened temporal arteries
29
Complications of GCA
Permanent blindness Stroke
30
Investigations for GCA
Bloods - ESR Temporal artery US Temporal artery biopsy CT angiogram / MR angiogram if still unsure
31
When should you suspect GCA in a patient
Patient over 50 years old, new onset of headache with raised inflammatory markers
32
What is important to be measured in bloods for GCA
ESR
33
What can be seen on temporal artery biopsy in GCA
Transmural inflammation of the layers of arteries Patchy infiltration by giant cells, lymphocytes Thickened vessel wall
34
Why does temporal artery biopsy have low sensitivity but high specificity for GCA
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
Management of GCA
Immediate Oral steroids - prednisolone Gradually reduce steroids dosage Steroid sparing agents if cannot reduce steroid dosage
36
What are the steroid sparing agents
Methotrexate Azathioprine Leflunamide
37
Why should you reduce the steroid dosage gradually instead of quickly
Because symptoms can relapse
38
Differences between takayasu's arteritis and GCA
Takayasu arteritis mainly affects the aorta whereas GCA affects temporal arteries Takayasu arteritis mainly affects younger patients whereas GCA affects older patients
39
Common biopsy finding between takayasu and GCA
skip lesions
40
What is ANCA associated small vessel vasculitis
Group of autoimmune inflammation conditions of small to medium vessels associated with anti-neutrophil cytoplasmic antibodies (ANCA)
41
What are the conditions part of ANCA vasculitis
Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis Microscopic polyangiitis
42
What is granulomatosis with polyangiitis
Granulomatous inflammation affecting small and medium sized vessels in the upper and lower resp tract, eyes, kidneys
43
Classic triad of granulomatosis with polyangiitis
Upper resp tract involvement Lower resp tract involvement Pauci-immune glomerulonephritis
44
What are the signs of URT involvement in granulomatosis with polyangiitis
Chronic sinusitis Epistaxis Saddle nose deformity Otitis media Mouth ulcers Sensorineural deafness
45
What are the signs of LRT involvement in granulomatosis with polyangiitis
Haemoptysis Cough Pleuritis Alveolar haemorrhage Bilateral cavitating nodules on CXR
46
What is pauci immune glomerulonephritis
Rapidly progressive condition leading to renal failure within days, strongly associated with ANCA
47
Presentation of pauci-immune glomerulonephritis
Proteinuria Haematuria Frothy urine due to proteinuria
48
What are the ocular involvement of granulomatosis with polyangiitis
Conjunctivitis Optic nerve vasculitis Retinal artery occlusion Uveitis Proptosis
49
What are the nervous system manifestations of GPA
mononeuritis multiplex polyneuropathy
50
What is mononeuritis multiplex
peripheral neuropathy of a single nerve at least 2 different areas of peripheral nervous system
51
Difference between mono neuritis multiplex and polyneuropathy
Polyneuropathy is damage to multiple nerves
52
What is eosinophilic granulomatosis with polyangiitis
granulomatous inflammation mostly affecting small and medium vessels in resp tract and skin
53
Diagnostic criteria of EGPA
Late onset adult asthma Eosinophilia Paranasal sinusitis Pulmonary infiltrates Histological confirmation of vasculitis Neuropathy - mononeuritis multiplex / polyneuropathy
54
Difference between granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis
EGPA has high eosinophil count EGPA causes late onset adult asthma whereas GPA doesn't
55
What is microscopic polyangiitis
Small to medium vessel vasculitis characterised by renal, pulmonary disease and pANCA
56
Which symptom is very common in microscopic polyangiitis
Necrotising glomerulonephritis
57
Symptoms of microscopic polyangiitis
Necrotising glomerulonephritis Palpable purpura Neuropathy Alveolar haemorrhage Haemoptysis
58
Investigations for ANCA- associated polyangiitis
Bloods Immunofluorescence Biopsy of affected areas
59
What may blood tests show in ANCA-associated polyangiitis
Raised inflammatory markers Anaemia Low complement level during active disease
60
What does immunofluorescence look at in ANCA associated polyangiitis
ANCA PR3 MPO
61
What are ANCA
autoantibodies against antigens in the cytoplasm of neutrophils
62
ANCA present in GPA
cANCA
63
ANCA present in EGPA
pANCA
64
ANCA present in MPA
pANCA
65
Can ANCA be diagnostic for ANCA associated polyangiitis
No, because a proportion of patients may be ANCA negative and the level of those autoantibodies vary with disease level
66
Management of ANCA associated polyangiitis depends on
involvement of organs - localised - early systemic - generalised - systemic - unresponsive
67
Localised ANCA associated polyangiitis =
only upper +/- lower resp tract involved
68
generalised ANCA associated polyangiitis =
Early renal involvement
69
Systemic ANCA associated polyangiitis =
Late renal involvement Vital organ failure
70
Management of localised and early systemic ANCA associated polyangiitis
Methotrexate + steroids
71
Management of generalised ANCA associated polyangiitis
Cyclophosphamide + steroids Azathioprine for maintenance
72
Management of systemic ANCA associated polyangiitis
Cyclophosphamide + steroids Plasma exchange if creatine > 500 Azathioprine for maintenance
73
Management of refractory ANCA associated polyangiitis
IV immunoglobulins Rituximab
74
What is Henoch-Schonlein Purpura
IgA mediated generalised vasculitis affecting small vessels of the skin, GI, kidneys, joints
75
Which age group is most commonly affected by Henoch-Schonlein Purpura
2-11 years old
76
Patients with Henoch-Schonlein Purpura usually had which preceding infections
Group A strep infection viral URTI pharyngeal infection GI infection
77
Symptoms of Henoch-Schonlein Purpura
Purpuric rash over buttocks and lower limbs Abdominal pain Bloody diarrhea Arthralgia Nephritis
78
Investigations for Henoch Schonlein Purpura
Clinical Urine dip stick Bloods
79
Management for Henoch Schonlein Purpura
Self limiting Regular urine dips for 12 months to check for renal impairment

Y2 MSK (19 decks)

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