Vasculitis Flashcards

1
Q

What is the definition of vasculitis?

A

Vessel wall inflammation

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2
Q

What is the pathophysiology of vasculitis?

A

Immune complex disease

Antibody dependent cellular cytotoxicity leads to endothelial activation and coagulopathy

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3
Q

Give 5 systemic symptoms of vasculitis

A
Malaise
Fever
Weight loss
Myalgia
Arthralgia
Sweats
Fatigue
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4
Q

Give 3 skin changes seen in vasculitis

A

Purpura
Ulceration
Infarction
Rash

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5
Q

Give 3 GI effects of vasculitis

A

Mouth ulcers
Abdo pain
Diarrhoea

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6
Q

Give 5 respiratory signs of vasculitis

A

Cough
Wheeze
Haemoptysis
Dyspnoea

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7
Q

Give 3 ENT signs of vasculitis

A

Epistaxis
Change of nose shape
Sinusitis
Deafness

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8
Q

What investigations would be done in someone where vasculitis is suspected?

A
Urinalysis
ESR
CRP
WCC
Biopsy 
Angiogram 
Organ specific tests
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9
Q

How is vasculitis managed?

A

Cyclophosphamide and high dose steroids to induce remission

To maintain remission, azathioprine, methotrexate, low dose steroids

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10
Q

Give 2 examples of large primary vasculitis

A

Takavasu’s arteritis
Giant cell arteritis
Polymyalgia rheumatica

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11
Q

Give 2 examples of medium primary vasculitis

A

Polyarteritis nodosa
Kawasaki disease
Isolated CNS vasculitis

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12
Q

Give 2 examples of small primary vasculitis

A

Wegeners granulomatosis (GPA)
EGPA
Microscopic polyangitis

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13
Q

Give 4 secondary causes of vasculitis

A
RA
SLE
Arthritis
Malignancy
Infection 
Drugs- cocaine
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14
Q

What common facial deformity is seen in Wegener’s granulomatosis (GPA)

A

Saddle nose deformity

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15
Q

What are the classic triad of systems involved in GPA?

A

Upper airway –> nasal obstruction, epistaxis, saddle nose

Lungs –> sinusitis, cough, chest pain, pleuritis, stridor

Kidneys –> glomerulonephritis, crescent formation, proteinuria

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16
Q

What investigations should be done if GPA is suspected?

A
ANCA (especially C-ANKA)
FBC- may have reduced Hb
Chest xray 
CT of sinuses
Lung biopsy
17
Q

Which vessels does Kawasaki’s disease mainly affect?

A

Medium vessels

Especially coronary vessels

18
Q

Why are patients with Kawasaki’s disease more at risk of leucocytosis and thrombocytosis?

A

Endothelial cells damaged so tunica media exposed which increases risk of coagulation (virchow’s triad)

19
Q

What antibody should be tested for in Kawasaki’s disease?

A

Anti-endothelial cell antibodies

20
Q

Give some signs and symptoms of Kawasaki’s disease?

A
CRASH and BURN
Conjunctivitis
Rash
Adenopathy 
Strawberry tongue
Hands and feet redness and oedema 

Burn = fever

21
Q

What is the main cause of death in Kawasaki’s disease?

A

Myocardial Infarction

22
Q

How is Kawasaki’s disease treated?

A

IV immunoglobulin

High dose aspirin (at risk of Reyes disease which is a rapidly progressing encephalopathy)

23
Q

What is Takayasu’s arteritis also known as?

A

Pulselessness disease

Aortic arch syndrome

24
Q

What vessels does Takayasu’s arteritis affect?

A

Large vessels

25
Q

Give 5 signs/symptoms in Takayasu’s arteritis

A
Absent peripheral pulses
Hypertension 
Heart failure 
Peripheral claudication 
Neurological symptoms 
Stroke
26
Q

How would Takayasu’s be investigated?

A

Contrast Angiography
CT
MRI
(No autoantibodies associated)

27
Q

How is Takayasu’s arteritis treated?

A

Corticosteroids

Surgical bipass

28
Q

What type of vessels does polyalgia rheumatica effect?

A

Large vessels

29
Q

What are the characteristic symptoms of polyalgia rheumatica?

A

Sudden onset pain and stiffness in shoulders and pelvis
Normal muscle strength
Worse in the morning and improves on exercise

Tiredness
Fever
Weight loss
Depression 
Night sweats
Loss of appetite
30
Q

What investigations should be done in polyalgia rheumatica?

A
ESR/CRP
FBC- normocytic anaemia
LFTs - may have raised ALP 
Temporal artery biopsy 
CK
Muscle biopsy
31
Q

How is polyalgia rheumatica treated?

A

Corticosteroids

32
Q

What are the symptoms of giant cell arteritis?

A
Headache- abrupt onset (over the temples) 
Scalp tenderness
Jaw claudication 
Sudden loss of vision 
Malaise
Tiredness
Fever
Thickened temporal arteries
(plus symptoms of polymyalgia rheumatica)
33
Q

How should giant cell arteritis be investigated?

A
Temporal artery biopsy 
FBC- normocytic anaemia 
ESR
CRP 
LFTs
34
Q

How is giant cell arteritis managed?

A

Early treatment
Corticosteroids
Osteoporosis prophylaxis
Methotrexate

35
Q

What is Behcet’s disease?

A

Mainly seen in Turkey, Iran and Japan
Unknown cause
Systemic vasculitis

36
Q

What are some of the signs and symptoms of Behcet’s disease?

A
Oral ulcers
Genital ulcers
Eye lesions 
Skin lesions 
Peripheral arthritis 
GI symptoms 
Leg thrombophlebitis
37
Q

How is Behcet’s investigated?

A

Pethergy reaction- skin test
ESR and CRP raised
No specific autoantibodies

38
Q

How is Behcet’s disease treated?

A
Corticosteroids 
Immunosuppressants 
Ciclosporin 
Colchicine 
Anti-TNFs (serious cases)