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11. Orthopaedics > Vasculitis - Primary Vasculitis > Flashcards

Vasculitis - Primary Vasculitis Flashcards

1
Q

List 2 types of large vessel vasculitis.

A

Temporal arteritis

Takayasu’s disease

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2
Q

What sort of patients are affected by temporal arteritis?

What about Takayasu’s disease?

A

Temporal arteritis: elderly women

Takayasu’s: young women

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3
Q

Describe the clinical features of temporal arteritis. (6)

A 
General inflammation
Thickened, visible temporal a.
Tenderness in temples/scalp
Headache
Fatigue
Jaw claudication
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4
Q

What specific test would you do for temporal arteritis?

Describe the results. (3)

A

Biopsy

Features:

  • Small lumen of artery
  • Thickened vessel walls
  • Inflammatory infiltrate
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5
Q

List 7 complications of temporal arteritis.

A

Affects nearby vessels (e.g. ophthalmic a., internal carotid a.)
Amaurosis fugax (sudden, painless blindness)
Tongue paraplesia
Sensorineural deafness
Stroke
Involvement of aorta
Coronary ischaemia

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6
Q

List 1 condition which is associated with temporal arteritis.

A

Polymyalgia rheumatica

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7
Q

How would you manage temporal arteritis?

A

Steroids (oral prednisolone)

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8
Q

Describe the clinical features of Takayasu’s disease. (7)

A 
SYMPTOMS:
Syncope
Facial muscle paralysis
Transient blindness
Claudication
Systemic features

SIGNS:
Absent pulses
BP variable between limbs

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9
Q

Which investigations would you do for Takayasu’s disease? (2)

A

Angiography

MRI

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10
Q

Define Takayasu’s disease.

What are 2 other names for this condition?

A

Pulseless disease
Aortic arch syndrome

Def.: “Progressive occlusion of the arteries arising from the arch of the aorta, resulting in absent pulses in arms/neck”

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11
Q

How would you treat Takayasu’s disease? (4)

A

Steroids (oral prednisolone)
BP control
Methotrexate/cyclophosphamide
Angioplasty/stenting (for critical stenosis)

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12
Q

List 2 examples of medium vessel vasculitis.

A

Polyarteritis nodosa

Kawasaki’s disease

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13
Q

Define polyarteritis nodosa.

A

Connective tissue disorder of unknown cause in which there is patchy inflammation of the artery walls

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14
Q

Describe the clinical features of polyarteritis nodosa. (3)

A

Systemic features, e.g.

  • Fever
  • Weight loss
  • Night sweats

Organ infarction

Organ-specific symptoms

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15
Q

Describe the consequences of polyarteritis nodosa in:

a) Kidneys (3)
b) Gut (1)
c) CV system (3)
d) MSK system (2)
e) Skin (2)

A

KIDNEYS:
Renal artery stenosis
Renal aneurysm
Hypertension

GUT:
Bowel infarction

CV SYSTEM:
Stroke
MI
Hypertension

MSK SYSTEM:
Arthritis
Critical limb ischaemia

SKIN:
Gangrene
Purpura

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16
Q

Define Kawasaki disease.

A

Condition of unknown cause affecting children <5 yo, characterised by fever, conjunctivitis, sore throat, generalised rash and reddening of palms/soles

17
Q

Describe the clinical features of Kawasaki disease. (8)

A

Large, pulsating aneurysms in several arteries
High fever

Other features, e.g.

  • Mucositis
  • Conjunctivitis
  • Strawberry tongue
  • Nail changes
  • Palmar erythema
  • Desquamation
18
Q

Describe the natural progression of Kawasaki disease.

HINT: there are 2 phases.

A

ACUTE PHASE (10 days)

  • Good prognosis
  • Presentation: signs of infection

SUBACUTE PHASE (10+ days)

  • Worse prognosis
  • Presentation: asymptomatic aneurysms; often only detected when they rupture and baby dies
19
Q

List 3 examples of immune complex mediated small vessel vasculitis.

A

Goodpasture’s syndrome
Cryoglobulinaemia
Henoch-Schonlein Purpura (HSP)

20
Q

Define Henoch-Schonlein purpura (HSP).

A

Common, recurrent form of purpura usually found in young children, characterised by red weals and a purple rash on the buttocks/lower legs due to bleeding into the skin from inflamed capillaries

21
Q

Describe the clinical features of HSP. (5)

What is the classical HSP triad?

A

HSP TRIAD:
Palpable purpura
Abdominal pain
Arthritis

Distribution of purpura:

  • Extensor surfaces
  • Starts peripherally, spreads proximally
22
Q

Describe the pathophysiology of HSP. (2)

A
  1. Unrelated infection stimulates formation of IgA deposits in small blood vessels
  2. This causes small vessel vasculitis
23
Q

List 7 complications of HSP.

A

KIDNEYS:
Nephritic syndrome

BOWEL:
Intussusception
Bowel infarction
Purpura (doughnut sign on colonoscopy)

EMOTIONS:
Pain and misery

TESTES:
Testes regression
Infarction

24
Q

How would you manage HSP? (1)

A

Supportive treatment

25
Q

Define Wegener’s granulomatosis.

What type of disease is this?

A

Def.: “An autoimmune disease predominantly affecting the nasal passages, lungs and kidneys, characterised by the formation of necrotising granulomas in addition to pulmonary vasculitis

c-ANCA positive vasculitis

26
Q

Describe the clinical features of Wegener’s granulomatosis. (4)

A

Upper/lower respiratory tract involvement
Crescentic GN
Palate necrosis/erosion
Saddle deformity of nose

27
Q

How would you manage Wegener’s granulomatosis? (3)

A

Steroids
Cyclophosphamide
Azathioprine

28
Q

Define microscopic polyangiitis.

What kind of disease is this?

A

Def.: “An ill-defined autoimmune disease characterised by a systemic, pauci-immune, necrotising small vessel vasculitis without necrotising granulomatous inflammation”

MPO positive, p-ANCA positive vasculitis

29
Q

Describe the clinical features of microscopic polyangiitis. (8)

A 
Breathlessness
Kidney impairment/failure
Fatigue
Joint pain
Skin rashes
Vision changes
Impaired nerve function
Diarrhoea
30
Q

How would you treat microscopic polyangiitis? (6)

A 
Azathioprine
Cyclophosphamide
Methotrexate
Mycophenolate
Rituximab
Steroids
31
Q

Define Churg-Straus syndrome.

What kind of disease is this?

A

Def.: “A clinical syndrome comprising of severe asthma associated with an increased eosinophil count in the peripheral blood and eosinophilic deposits in the pulmonary vessels”

MPO positive, p-ANCA positive vasculitis

32
Q

Describe the clinical features of Churg-Strauss syndrome. (3)

A

Eosinophilia
Asthma
Widespread infiltrate in lungs

33
Q

How would you treat Churg-Strauss syndrome? (1)

A

Steroids

11. Orthopaedics (13 decks)

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