[10] Vasculitis Flashcards

1
Q

What are the classification of vasculitis?

A

Large vessel
Medium vessel
Small vessel

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2
Q

Give two examples of large vessel vasculitis

A

Giant Cell Arteritis

Takayasu’s arteritis

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3
Q

Give two examples of medium vessel vasculitis

A

Polyarteritis nodosa

Kawasaki disease

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4
Q

How can small vessel vasculitides be further classified?

A

pANCA
cANCA
ANCA -ve

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5
Q

Give two examples of cANCA small vessel vasculitis

A

Churg-Strauss

Microscopic polyangiits

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6
Q

Give an example of a cACNA small vessel vasculitis

A

Wegener’s granulomatosis

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7
Q

Give 4 examples of a ANCA -ve small vessel vasculitis

A

Henoch-Schonlein purpura
Goodpasture’s disease
Cryoglobulinaemia
Cutaneous leukocytoclastic vasculitis

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8
Q

What is giant cell arteritis also known as?

A

Temporal arteritis

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9
Q

Who is giant cell arteritis common in?

A

Elderly

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10
Q

Who is giant cell arteritis rare in?

A

<55

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11
Q

What % of cases of giant cell arteritis are associated with polymyalgia rheumatica?

A

50%

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12
Q

What are the features of giant cell arteritis?

A
Systemic signs
Headache
Temporal artery and scalp tenderness
Jaw claudication
Amaurosis fugax
Prominent temporal arteries, with or without pulsation
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13
Q

What are the systemic signs of giant cell arteritis?

A

Fever
Malaise
Fatigue

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14
Q

What action should be taken if you suspect a patient has giant cell arteritis?

A

Do an ESR, and start prednisolone 40-60mg/day PO

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15
Q

What may be found on blood tests in giant cell arteritis?

A

Greatly increased ESR and CRP
Increased ALP
Decreased Hb
Increased platelets

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16
Q

What kind of anaemia is present in giant cell arteritis?

A

Normocytic normochromic

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17
Q

How quickly should a temporal artery biopsy be done in suspected giant cell arteritis?

A

Within 3 days

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18
Q

How should giant cell arteritis be managed after starting steroids?

A

Taper steroids gradually

Give PPI and alendronate cover

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19
Q

What should the tapering of steroids be guided by in giant cell arteritis?

A

Symptoms and ESR

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20
Q

How long should PPI and alendronate be given in giant cell arteritis?

A

Usually 2 year course

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21
Q

When does polymyalgia rheumatica present?

A

> 50 years old

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22
Q

How does polymyalgia rheumatica present?

A

Severe pain and stiffness in shoulders, neck, and hips

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23
Q

Describe the onset of symptoms in polymyalgia rheumatica?

A

Sudden/subacute onset

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24
Q

Are the symptoms of polymyalgia rheumatica symmetrical?

A

Yes

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25
Q

When is the pain of polymyalgia rheumatica worse?

A

In the morning

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26
Q

Is there associated weakness in polymyalgia rheumatica?

A

No

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27
Q

What other features may be present in polymyalgia rheumatica?

A

Mild polyarthritis
Tenosynovitis
Carpal tunnel syndrome
Systemic signs

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28
Q

What systemic signs may be present in polymyalgia rheumatica?

A

Fatigue
Fever
Weight loss

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29
Q

What % of patients with polymyalgia rheumatica develop giant cell arteritis?

A

15%

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30
Q

What is found on investigation in polymyalgia rheumatica?

A

Increased ESR and CRP
Increased plasma viscosity
Increased ALP
Normal CK

31
Q

How is polymyalgia rheumatica managed?

A

Same as giant cell arteritis

32
Q

Where is Takayasu’s arteritis found?

A

Japan - rare outside of Japan

33
Q

What gender is more commonly affected wiht Takayasu’s arteritis?

A

Females

34
Q

What age is Takayasu’s arteritis most common in?

A

20-40 years

35
Q

What are the features of Takayasu’s arteritis?

A

Weak pulses in upper extremities
Visual disturbance
Hypertension
Constitutional symptoms

36
Q

What are the constitutional symptoms of Takayasu’s arteritis?

A

Fever
Fatigue
Weight loss

37
Q

Is polyarteritis nodosa common in UK?

A

No, is rare

38
Q

What it the male to female ratio of polyarteritis nodosa?

A

M:F = 2:1

39
Q

What age group is most commonly affected with polyarteritis nodosa?

A

Young adults

40
Q

What infection is polyarteritis associated with?

A

Hepatitis B

41
Q

What are the features of polyarteritis nodosa?

A

Rash
Hypertension
Melaena and abdominal pain
Constitutional symptoms

42
Q

How is polyarteritis nodosa managed?

A

Prednisolone and cyclophosphamide

43
Q

What is Kawasaki’s disease?

A

A childhood PAN variant

44
Q

What are the features of Kawasakis disease?

A
5-day fever
Bilat non-purulent conjunctivitis
Oral mucositis 
Cervical lymphadenopathy
Polymorphic rash
Extremity changes 
Coronary artery aneurysms
45
Q

What extremity changes are seen in Kawasaki’s disease?

A

Erythema and desquamation

46
Q

How is Kawasaki’s disease managed?

A

IVIg and aspirin

47
Q

What happens in Wegener’s granulomatosis?

A

Necrotising granulomatous inflammation and small vessel vasculitis, which particularly affects the URT, LRT, and kidneys

48
Q

What are the URT features of Wegener’s granulomatosis?

A

Chronic sinusitis
Epistaxis
Saddle-nose deformity

49
Q

What are the LRT features of Wegener’s granulomatosis?

A

Cough
Haemoptysis
Pleuritis

50
Q

What are the renal features of Wegener’s granulomatosis?

A

Rapidly progressive glomerulonephritis

51
Q

What are the skin features of Wegener’s granulomatosis?

A

Palpable purpura

52
Q

What are the ocular features of Wegener’s granulomatosis?

A

Conjunctivitis
Keratitis
Uveitis

53
Q

What investigations are done in Wegener’s granulomatosis?

A

Urine dipstick

CXR

54
Q

What are the features of Churg-Strauss?

A

Late-onset asthma
Eosinophilia
Granulomatous small-vessel vasculitis

55
Q

What does the granulomatous small-vessel vasculitis lead to in Churg-Strauss?

A

Rapidly progressive glomerulonephritis
Palpable purpura
GIT bleeding

56
Q

What autoantibody is found in Wegener’s granulomatosis?

A

cANCA

57
Q

What autoantibody is found in Churg-Strauss?

A

pANCA

58
Q

What drug might Churg-Strauss be associated with?

A

Montelukast

59
Q

What are the features of microscopic polyangiitis?

A

Rapidly progressive glomerulonephritis
Haemoptysis
Palpable purpura

60
Q

What autoantibody is found in microscopic polyangiitis?

A

pANCA

61
Q

What is produced in Goodpastures?

A

Anti-glomerular basement membrane antibodies

62
Q

What are the features of Goodpastures syndrome?

A

Rapidly progressive glomerulonephritis

Haemoptysis

63
Q

What is found on CXR in Goodpastures?

A

Bilateral lower zone infiltrates (haemorrhage)

64
Q

How is Goodpastures managed?

A

Immunosuppression and plasmapheresis

65
Q

What is produced in simple cryoglobulinaemia?

A

Monoclonal IgM

66
Q

What is simple cryoglobulinaemia secondary to?

A

Myeloma
CLL
Waldenstroms

67
Q

What does simple cryoglobulinaemia cause?

A

Hyperviscosity

68
Q

What does the hyperviscosity in simple cryoglobulinaemia lead to?

A
Visual disturbance
Bleeding from mucous membranes
Thrombosis
Headaches
Seizures
69
Q

What % of cases of cryoglobulinaemia are mixed?

A

80%

70
Q

What is produced by mixed cryoglobulinaemia?

A

Polyclonal IgM

71
Q

What is mixed cryoglobulinaemia secondary to?

A

SLE
Sjorgens
Hep C
Mycoplasma

72
Q

What does mixed cryoglobulinaemia lead to?

A

Immune complex disease

73
Q

What does the immune complex disease in mixed cryoglobulinaemia lead to?

A

Glomerulonephritis
Palpable purpura
Arthralgia
Peripheral neuropathy