Vasculitis Flashcards

1
Q

What is vasculitis?

A

inflammation of blood vessels

often with ischaemia/necrosis/organ inflammation

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2
Q

What type of blood vessel is affected by vasculitis?

A

can affect any blood vessel

=> arteries, arterioles, veins, venules, or capillaries

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3
Q

What is the difference between primary and secondary vasculitis?

A

Primary - autoimmune inflammatory response targets vessel walls and has no known cause

Secondary - triggered by an infection/drug/toxin OR another inflammatory disorder or cancer.

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4
Q

Describe the pathogenic changes that occur inside the blood vessel walls?

A
activated macrophages produce mediators
progressive vascular inflammation
endothelial damage
disruption of internal elastic lamina
intimal hyperplasia
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5
Q

What vessels are affected in small vessel vasculitis?

A

arterioles, capillaries and venules

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6
Q

What vessels are affected in medium vessel vasculitis?

A

arteries

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7
Q

Give examples of conditions which are classed as small vessel vasculitis

A

Cryoglobulinemic
IgA mediated
Hypocomplementemic urticarial

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8
Q

Give examples of medium vessel vasculitis

A

Polyarteritis nodosa

Kawasaki Disease

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9
Q

What type of vasculitis are Giant Cell and Takayasu arteritis?

A

Large vessel

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10
Q

What types of small vessel vasculitis are ANCA associated?

A

Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis

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11
Q

What symptoms are common to all types of vasculitis?

A

fever
malaise
weight loss
fatigue

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12
Q

What age group and gender are more likely to get Takayasu arteritis?

A

<40 years and commoner in females.

More prevalent in Asian populations.

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13
Q

What age group is most likely to get giant cell arteritis?

A

> 50 years

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14
Q

What features commonly present in large vessel vasculitis?

A

Bruit (usually carotid artery)
Blood pressure difference of extremities
Claudication

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15
Q

What other muscular condition is associated with temporal arteritis?

A

polymyalgia rheumatica

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16
Q

What symptoms are usually present in temporal arteritis?

A

Unilateral temporal headache
scalp tenderness
jaw claudication

Temporal arteries prominent with reduced pulsation.

17
Q

What nerve is at risk of being damaged in temporal arteritis and what are the consequences?

A

Risk of blindness due to ischaemia of the optic nerve.

18
Q

What investigations are used to diagnose temporal arteritis?

A

ESR, plasma viscosity and CRP raised

Temporal artery biopsy
(“skip lesions” occur so biopsy may be negative)

MR angiogram or PET CT

19
Q

What treatment is used for temporal arteritis?

A

40-60mg prednisolone.

Steroid sparing agents may be considered.

20
Q

What condition is characterised by granulomatous inflammation of the respiratory tract, small and medium vessels with common necrotising glomerulonephritis?

A

granulomatosis polyangiitis

21
Q

What condition is characterised by granulomatous inflammation of respiratory tract and small/medium vessels and an association with asthma?

A

Eosinophilic granulomatosis with polyangiitis

22
Q

Necrotising vasculitis with a few immune deposits, and necrotising glomerulonephritis is common in this condition.

A

Microscopic polyangiitis

23
Q

What age group and what gender most commonly develop granulomatosis polyangiitis ?

A

Male-to-female ratio of 1.5 : 1

Typically age 35-55 years

24
Q

What organ systems are involved in granulomatosis polyangiitis?

A
ENT
Respiratory
Skin
Renal
Nervous system
Ocular
25
Q

What are the criteria for a diagnosis of eosinophilic granunlomatosis with polyangiitis?

A

Asthma
Eosinophilia (>10% in peripheral blood)
Paranasal sinusitis
Pulmonary infiltrates (may be transient)
Histological proof of vasculitis with extravascular eosinophils
Mononeuritis multiplex or polyneuropathy

must have 4 or more

26
Q

What are ANCA antibodies?

A

Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)

auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes

27
Q

What test is used to detect ANCA?

A

Immunofluoresence
Can differentiate ANCA patterns

e.g. cANCA and pANCA

28
Q

c-ANCA is associated with GPA and EGPA, whilst p-anca is associated with MPA. TRUE/FALSE?

A

FALSE
c-ANCA is associated with GPA
p-ANCA is associated with EGPA and MPA

29
Q

Is PR3 or MPO linked to GPA?

A

PR3

MPO is high in EGPA and MPA

30
Q

What happens to complement levels when disease is active?

A

Complement is consumed during active disease so C3/4 may fall

31
Q

How is ANCA Associated Vasculitis managed?

A

Local/early systemic = Methotrexate + steroids

Generalised/systemic = Cyclophosphamide + steroids

Refractory = IV immunoglobulins / Ritixumab

32
Q

What microbe usually causes the infection which precedes HSP?

A

group A streptococcus

33
Q

What are the common symptoms of HSP?

A

Purpuric rash typically over buttocks and lower limbs

Colicky abdominal pain

Bloody diarrhoea

Joint pain +/- swelling

Renal involvement (50%)

34
Q

Is HSP self limiting?

A

Usually self-limiting

Symptoms tend to resolve within 8 weeks