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MSK- Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels

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2
Q

What can the inflammation in vasculitis result in?

A

Vessel wall thickening, stenosis and occlusion with subsequent ischaemia, necrosis and organ inflammation

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3
Q

What blood vessels can vasculitis affect?

A

Any (i.e. artery, vein, capillary)

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4
Q

What system does vasculitis affect?

A

It is a multi-system disease

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5
Q

What is the mortality rate of small vessel vasculitis if left untreated for 2 years?

A

90%

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6
Q

What is primary vasculitis?

A

Results from an inflammatory response that targets the vessel walls and has no known cause (can be autoimmune)

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7
Q

What is secondary vasculitis?

A

May be triggered by an infection, drug or toxin or as part of another inflammatory disorder or cancer

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8
Q

What cell mediates vasculitis?

A

T cells

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9
Q

How are small vessel vasculitis’ split into two categories?

A

ANCA positive ANCA negative

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10
Q

If there is ANCA + vasculitis, and granulomas are present, what could this be?

A

EGPA or GPA

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11
Q

If there is ANCA + vasculitis and there are no granulomas present, what would this be?

A

MPA

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12
Q

What are some systemic features of vasculitis which may be present in all types?

A
  • Fever - Malaise - Weight loss - Fatigue - Night sweats - Arthralgia
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13
Q

What is large vessel vasculitis?

A

Primary vasculitis which causes chronic granulomatous inflammation, predominantly of the aorta and its major branches

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14
Q

What are the two types of large vessel vasculitis?

A
  • Takayusu Arteritis - Giant Cell (Temporal) Arteritis
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15
Q

Which arteries are often affected in Takayusu Arteritis?

A

Aorta, femoral and subclavian arteries

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16
Q

Who does Takayusu Arteritis normally affect?

A

Those < 50, predominantly women in their 2nd-3rd decade

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17
Q

Which population is Takayusu Arteritis most common in?

A

Asians

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18
Q

Who does Giant Cell Arteritis usually affect?

A

> 50s

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19
Q

What arteries are affected by Giant Cell Arteritis?

A

Usually the temporal artery, but the aorta and other large vessels can be involved

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20
Q

What can occur if large vessel vasculitis is left untreated?

A

Vascular stenosis and aneurysms which can cause reduced pulses and bruits

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21
Q

What are 5 presenting features of large vessel vasculitis?

A
  • Bruit - BP difference in extremities - Claudication - Carotodynia or vessel tenderness - Hypertension
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22
Q

Where is a bruit most commonly found in large vessel vasculitis?

A

Carotid arteries

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23
Q

What disease does temporal arteritis have an association with?

A

Polymyalgia rheumatica

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24
Q

What is the major risk of temporal arteritis?

A

Blindness due to ischaemia of the optic nerve

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25
Q

What are some investigations for large vessel vasculitis? What will they show?

A
  • Inflammatory markers will be raised - MR angiogram (may show thickening, stenosis or aneurysm) - PET CT (shows increased metabolic activity of inflamed vessels)
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26
Q

What investigation is done if there is suspected temporal arteritis? Is this always sensitive?

A

Temporal artery biopsy- there are skip lesions so can show up negative even when the condition is present

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27
Q

What is the treatment for large vessel vasculitis?

A

40-60mg prednisolone, gradually reducing dose Can use steroid sparing agents such as azathioprine or methotrexate

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28
Q

What are the two types of medium vessel vasculitis?

A

Kawasaki Disease Polyarteritis Nodosa

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29
Q

Who is Kawasaki Disease seen in?

A

Children, mainly < 5 years

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30
Q

Which vessels does Kawasaki Disease affect?

A

Various ones, the most important being the coronary arteries where aneurysms can develop

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31
Q

What characterises Polyarteritis Nodosa?

A

Necrotising inflammatory lesions that affect arteries at vessel bifurcations, resulting in aneurysms

32
Q

Which sites does Polyarteritis Nodosa affect?

A

Skin, gut, kidneys

33
Q

Polyarteritis Nodosa is associated with what other disease?

A

Hepatitis B

34
Q

Describe the pathology of GPA (Wegener’s)?

A

Granulomatous inflammation of the respiratory tract, small and medium vessels. Necrotising glomerulonephritis is common.

35
Q

Describe the pathology of EGPA (Churg-Strauss)?

A

Eosinophilic granulomatous inflammation of the respiratory tract, small and medium vessels. Associated with late onset asthma.

36
Q

Describe the pathology of MPA?

A

Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis is very common.

37
Q

Where does MPA only really affect?

A

The kidneys

38
Q

GPA is more common in individuals from where?

A

North Europe

39
Q

What sex is GPA more common in?

A

Males, but not by much

40
Q

What is the age onset of GPA?

A

Can occur at any age, but typically 35-55 years

41
Q

A person has GPA if two of what four features are present?

A
  • Nasal or oral inflammation - Abnormal chest radiograph - Urinary sediment - Granulomatous inflammation on biopsy
42
Q

What are some ENT features of GPA?

A

Sinusitis, nasal crusting, epistaxis, mouth ulcers, deafness, saddle nose

43
Q

What causes saddle nose in GPA?

A

Cartilage ischaemia

44
Q

What are some respiratory features of ANCA vasculitis?

A

Pulmonary infiltrates, cough, haemoptysis, nodules on x-ray

45
Q

What are some cutaneous features of ANCA vasculitis?

A

Non-blanching purpura, cutaneous ulcers

46
Q

What does necrotising glomerulonephritis manifest as?

A

Blood and protein in the urine

47
Q

What are some neurological features of ANCA vasculitis?

A

Mononeuritis multiplex, polynephropathies, cranial nerve palsies

48
Q

What is mononeuritis multiplex? Which ANCA vasculitis is it more common in?

A

A peripheral nerve stops working because of a lack of blood flow- more common in EGPA

49
Q

What are some ocular features of ANCA vasculitis?

A

Conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis

50
Q

What is the main difference in the presentation of EGPA compared with GPA?

A

Late onset asthma, high eosinophil count, rhinitis

51
Q

Eosinophilia must be of what percent in the blood to diagnose EGPA?

A

> 10%

52
Q

What is ANCA?

A

Antibodies against antigens in the cytoplasm of neutrophil granulocytes

53
Q

Does every patient with GPA, EGPA, MPA have ANCA antibodies?

A

No

54
Q

What test is used to detect ANCA?

A

Immunofluorescence

55
Q

cANCA and PR3 suggest what disease?

A

GPA

56
Q

pANCA and anti-MPO suggest what disease?

A

EGPA

57
Q

Which is more specific, cANCA or pANCA?

A

cANCA

58
Q

Why can cANCA, pANCA, PR3 and anti-MPO be useful monitoring tools?

A

They vary with disease activity

59
Q

ANCA associated vasculitis is associated with the formation of what?

A

Immune complexes

60
Q

What happens to complement levels during disease activity?

A

Decreased

61
Q

What is the most common treatment for ANCA associated vasculitis, especially if there is evidence of renal disease?

A

IV steroids and cyclophosphamide

62
Q

What would be the treatment for localised ANCA vasculitis with no systemic involevement, or early systemic involvement?

A

Methotrexate/azathioprine + steroids

63
Q

If cyclophosphamide and steroids do not work as a treatment for ANCA vasculitis, what is the next step?

A

Rituximab and steroids

64
Q

What treatment is used in ANCA vasculitis if creatinine is > 500?

A

Plasma exchange

65
Q

What is used for ANCA vasculitis treatment for very progressive disease, unresponsive to all other treatments?

A

IV immunoglobulins and rituximab

66
Q

What investigations are used for ANCA vasculitis?

A
  • Inflammatory markers (raised) - Anaemia - U+Es and urinalysis to look for renal involvement - ANCA - CXR - Biopsy of affected area
67
Q

What is an example of a non-ANCA small vessel vasculitis?

A

Henoch Schonlein purpura

68
Q

What mediates Henoch Schonlein purpura?

A

IgA

69
Q

Who does Henoch Schonlein purpura usually occur in?

A

Children

70
Q

What triggers Henoch Schonlein purpura?

A

A preceding infection, usually URTI caused by group A strep, though can be a GI infection

71
Q

How long does the preceding illness usually predate the onset of Henoch Schonlein purpura?

A

1-3 weeks

72
Q

How does Henoch Schonlein purpura present?

A

A purpuric rash over the buttocks and lower limbs, can also have colicky abdominal pain, joint pain, bloody diarrhoea, renal involvement

73
Q

How long does Henoch Schonlein purpura last for?

A

This is a self-limiting condition which usually resolves in 8 weeks but can relapse

74
Q

What should you always do when someone presents with Henoch Schonlein purpura?

A

Urinalysis for renal involvement

75
Q

What type of ANCA is this?

A

cANCA

76
Q

What type of ANCA is this?

A

pANCA

MSK- Rheumatology (12 decks)

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