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
What are the criteria for a diagnosis of eosinophilic granunlomatosis with polyangiitis?
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
What are ANCA antibodies?
Anti-Neutrophil Cytoplasmic Antibodies (ANCAs) auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes
27
What test is used to detect ANCA?
Immunofluoresence Can differentiate ANCA patterns e.g. cANCA and pANCA
28
c-ANCA is associated with GPA and EGPA, whilst p-anca is associated with MPA. TRUE/FALSE?
FALSE c-ANCA is associated with GPA p-ANCA is associated with EGPA and MPA
29
Is PR3 or MPO linked to GPA?
PR3 MPO is high in EGPA and MPA
30
What happens to complement levels when disease is active?
Complement is consumed during active disease so C3/4 may fall
31
How is ANCA Associated Vasculitis managed?
Local/early systemic = Methotrexate + steroids Generalised/systemic = Cyclophosphamide + steroids Refractory = IV immunoglobulins / Ritixumab
32
What microbe usually causes the infection which precedes HSP?
group A streptococcus
33
What are the common symptoms of HSP?
Purpuric rash typically over buttocks and lower limbs Colicky abdominal pain Bloody diarrhoea Joint pain +/- swelling Renal involvement (50%)
34
Is HSP self limiting?
Usually self-limiting | Symptoms tend to resolve within 8 weeks