Vasculitis Flashcards

(38 cards)

1
Q

What is vasculitis?

A

An inflammatory blood vessel disorder

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2
Q

What are the small vessel vasculitis + their associated autoantibodies?

A
  • microscopic polyangiitis - p-ANCA
  • granulomatosis with polyangiitis - c-ANCA
  • eosinophilic granulomatosis with polyangiitis - p-ANCA + raised eosinophils
  • IgA vasculitis
  • Goodpasture’s syndrome (anti-glomerular basement membrane disease
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3
Q

What are the medium vessel vasculitis?

A

Polyarteritis nodosum
Kawasaki disease

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4
Q

What are the large vessel vasculitis?

A

Giant cell arteritis
Takayasu’s arteritis

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5
Q

what are the ANCA associated vasculitis?

A
  • granulomatosis with polyangiitis - c-ANCA
  • microscopic polyangitis - p-ANCA
  • eosinophilic granulomatosis with polyangitis - p-ANCA
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6
Q

common findings in ANCA associated vasculitis

A
  • renal impairment > raised creatinine, haematuria + proteinuria
  • dyspnoea
  • haemoptysis
  • fatigue
  • weight loss
  • fever
  • sinuitis
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7
Q

investigations on ANCA assocaited vasculitis

A
  • urinalysis
  • bloods - FBC, U&E, ANCA testing
  • chest x ray
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8
Q

What may be seen on an FBC in ANCA-associated vasculitis?

A

normocytic anaemia
thrombocytosis

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9
Q

What is associated with cANCA?

A

granulomatosis with polyangiitis

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10
Q

What is associated with pANCA?

A
  • eosinophilic granulomatosis with polyangiitis
  • microscopic polyangitis
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11
Q

Treatment of ANCA associated vasculitis

A
  • induction + maintenance phases
  • induction: high dose prednisolone combined with rituximab or cyclophosphamide
  • maintenance: azathioprine, methotrexate
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12
Q

What type of vessel does IgA vasculitis affect?
What are the key features?

A

Small vessel
Purpura
IgA nephritis

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13
Q

What type of vessel does microscopic polyangiitis affect?
What are the key features?

A
  • Small blood vessels
  • Glomerulonephritis > haematuira + proteinuria
  • Diffuse alveolar haemorrhages > haemoptysis
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14
Q

What type of vessel does granulomatosis with polyangiitis affect?
What are the key features?

A
  • Small blood vessels
  • Nasal + respiratory symptoms > epistaxis, sinusitis, dypnoea, haemoptysis, saddle shape nose deformity
  • Glomerulonephritis
  • c-ANCA
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15
Q

What type of vessel does eosinophilic granulomatosis with polyangiitis affect?
What are the key features?

A
  • Small blood vessels
  • Late-onset asthma
  • Sinusitis + dyspnoea
  • p-ANCA
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16
Q

What type of vessel does polyarteritis nodosa affect?
What are the key features?

A
  • Medium sized blood vessels
  • Renal impairment
  • Hypertension
  • Cardiovascular events - aneurysms
  • levido reticularis
17
Q

What type of vessel does Kawasaki disease affect?
What are the key features?

A
  • Medium sized blood vessels
  • widespread rash
  • high fever for >5/7
  • bilateral conjunctivitis
  • strawberry tongue
  • coronary artery aneurysms
  • in children
18
Q

Management of kawaski disease

A
  • high dose aspirin
  • IV immunoglobulin
  • echo to screen for conoary artery aneurysms
19
Q

Why is aspirin normally contraindicated in children

A

Risk of Reye’s syndrome

20
Q

What is Reye’s syndrome?

A
  • severe, progressive encephalopathy affecting children
  • accompanied with fatty infiltration of liver, kidneys + pancreas
21
Q

Features of Reye’s syndrome

A
  • peak at 2 years old
  • Hx of proceeding viral illness
  • encephalopathy > confusion, seizures, coma
  • fatty infiltration of liver, pancreas + kidneys
  • hypoglycaemia
22
Q

What type of vessel does giant cell arteritis affect?
What are the key features?

A
  • Large blood vessels
  • Unilateral headache
  • Scalp tenderness
  • Visual disturbance
  • Jaw claudiation
23
Q

What is the cause of ocular complications in GCA?

A

anterior ischaemic optic neuropathy
due to occulision of ophthalmic artery > ischaemia of optic nerve head

24
Q

Fundoscopy findings in GCA

A

swollen pale disc + blurred margains

25
Treatment of GCA
- give as soon as diagnosis is suspected + before temporal artery biopsy - if no visual loss - **high dose prednisolone** - if visual loss - **IV methylprednisolone before high dose oral prednisolone** - bisphosphonates whilst on steroids
26
What type of vessel does Takayasu arteritis affect? What are the key features?
- Large blood vessels - Aortic arch affected - absent limb pulse - unequal BP in upper limbs - upper + lower limb claudication on exertion - associated with renal artery stenosis
27
investigations of takayasu's arteritis
MR angiography or CT angiography
28
Treatment of takaysau's arteritis
systemic steroids
29
Investigations of vasculitis
- **raised inflammatory markers** *e.g. CRP+ESR* - ANCA (anti-neutrophil cytoplasmic antibodies) - **urine dipstick** for renal involvement
30
Management of vasculitis
- rule out infection - stop offending drugs in secondary causes - 1st line: **corticosteroids** *e.g. oral prednisolone* - 2nd line: **biological agents or immunomodulatory drugs** *e.g. cyclophosphamide, rituximab, methotrexate*
31
How do clinical features arise in vasculitis?
Due to damage of blood vessel walls with subsequent thrombosis, ischameia, bleeding and/or aneurysm formation
32
What is Behcet disease?
Complex inflammatory condition affecting blood vessels + tissues
33
Main features of Behçet’s disease
- recurrent oral + genital ulcers - anterior uveitis - painful, sharply circumscribed erosions with red halo - heal over 2-4 weeks - abdo pain, diarrhoea
34
Management of Behçet’s disease
- **topical steroids** for mouth ulcers *e.g. soluble betamethasone* - **systemic steroids** - *colchicine* - **topical anaesthetics** for genital ulcers *e.g. lidocaine ointment*
35
What is Goodpasture’s syndrome (anti-glomerular basement membrane disease)
- rare small vessel vasculitis - associated with pulmonary haemorrhage + rapidly progressive glomerulonephritis - caused by anti-GBM antibodies against type IV collagen
36
Features of anti-GBM disease/Goodpasture’s syndrome-
- pulmonary haemorrhage - rapidly progressive glomerulonephritis > AKI, nephritic syndrome > proteinuria + haematuria
37
Investigations of anti GBM disease/Goodpasture’s syndrome
- renal biospy > liner IgG deposits in basement membrane - raised transfer factor due to pulmonary haemorrhage
38
Management of anti-GBM/Goodpasture syndrome
- plasmapheresis - steroids - cyclophosphamide