Vasculitis 11/10/18 Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels, often with ischaemia, necrosis and organ inflammation

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2
Q

Where can vasculitis affect?

A
Any blood vessels
Arteries
Arterioles
Veins
Venules
Capillaries
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3
Q

What is the mortality rate for untreated small vessel vasculitis?

A

90% after 2 years

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4
Q

What are the two types of vasculitis?

A

Primary

Secondary

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5
Q

What is primary vasculitis?

A

From inflammatory response that targets the vessel walls and has no known cause
Can be autoimmune

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6
Q

What is secondary vasculitis?

A

May be triggered by infection, a drug, or a toxin

May occur as part of another inflammatory disorder or cancer

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7
Q

Diagram on SLide 7

A

xoxo

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8
Q

What is vasculitis classified by?

A

What size of vessel is affected

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9
Q

What sizes of vessels can be affcted and what are examples?

A
Large vessel vasculitis
-Takayasu arteritis
-Giant cell arteritis
Medium vessel vascullitis
- Kawasaki disease
Small vessel
-ANCA associated
-Immune complex
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10
Q

What systemic symptoms may be experienced with all types of vasculitis?

A

Fever
Malaise
Weight loss
Fatigue

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11
Q

What are the two main causes of large vessel vasculitis?

A

Takayasu arteritis

Giant Cell arteritis

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12
Q

Who gets TA?

A

<40
Females
Asian

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13
Q

Who gets GCA?

A

Over 50s

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14
Q

What vessels are affected in GCA?

A

Temporal

Aorta and other large vessels can be affected

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15
Q

What is seen in large vessel vasculitis?

A

Granulomatous infiltration of the walls of the large vessels

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16
Q

What are the presenting features in LVV?

A

Bruit (carotid)
Blood pressure difference in limbs
Claudication

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17
Q

What is Temporal arteritis associated with?

A

Polymyalgia rheumatica

18
Q

What symptoms are seen in temporal arteritis?

A
Headache
Scalp tenderness
Jaw claudication
Prominent temporal arteries
Reduced Pulsation
Risk of blindness
19
Q

What investigations are done for Temporal arteritis?

A
ESR
Plasma viscosity
Temporal artery biopsy
CRP
MR angiogram
PET CT
20
Q

What is the management of TA/LVV?

A

40-60mg prednisolone

Steroid sparing agents

21
Q

What are the two branches of small vessel vasculitis?

A

ANCA associated

ANCA negative

22
Q

Who gets GPA?

A

Males
Northern european
35-55

23
Q

What are the ENT features of GPA?

A
Sinusitis
Nasal crusting
Epistaxis
Mouth ulcers
Sensorineural deafness
Otitis media
Saddle nose
24
Q

What are the Respiratory features of GPA?

A
Pulmonary infiltrates
COugh
Haemoptysis
Diffuse alveolar haemorrhage
Cavitating nodules
25
Q

What are the cutaneous features of GPA?

A

Palpable purpura

CUtaneous ulcers

26
Q

What are the renal features of GPA?

A

Necrotising glomerulonephritis

27
Q

What are the nervous system features of GPA?

A

Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies

28
Q

What are the ocular features of GPA?

A
Conjunctivitis
Episcleritis
Uveitis
Optic nerve vasculitis
Retinal artery occlusions
Proptosis
29
Q

What is the main difference between EGPA and GPA?

A

Late onset asthma

High eosinophil count

30
Q

ACR criteria

A
Asthma
Eosinophillia
Paranasal sinusitis
Pulmonary infiltrates
Proof of vasculitis with eosinophils
Polyneuropathy
31
Q

Immunology

A

ANCAs

Immunoflourescence

32
Q

What are the ocular features of GPA?

A
Conjunctivitis
Episcleritis
Uveitis
Optic nerve vasculitis
Retinal artery occlusions
Proptosis
33
Q

How is localised AAV treated?

A

Methotrexate + steroids

34
Q

How is generalised AAV treated?

A

Steroids +
-Cyclophosphamise
Rituximab
Followed by

35
Q

How is generalised AAV treated?

A
Steroids +
-Cyclophosphamise
Rituximab
Followed by:
Azathiprine
36
Q

How is refractory AAV treated?

A

Iv immunoglobulins

Rituximab

37
Q

How is refractory AAV treated?

A

Iv immunoglobulins

Rituximab

38
Q

What is Henoch-Schonlein purpura?

A

An acute immunoglobulin A mediated disorder

39
Q

What is the presentation of HSP?

A
Purpuric rash typically over buttocks and lower limbs
Colicky abdo pain
Bloody diarrhoea
Joint pain
Renal involvement
Generalised vasculitis
Small vessels of the skin
Gi tract
Kidneys
Joints
Lungs
Children
40
Q

What is HSP caused by?

A

Group A strep

41
Q

How is HSP managed?

A

Self-limiting
Symptoms tend to resolve within 8 weeks
Relapse may occur
Essentiial to perform urinalysis to screen for renal involvement