Vasculitis Flashcards

Dr J Golla

1
Q

what are the core clinical problems seen in vasculitis?

A
joint pain/swelling
abnormal bloods
rash
red/painful eye
SOB
cough
heaache
vision loss
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2
Q

define vasculitis

A

inflammation of blood vessels, often with ischemia, necrosis, and organ inflammation

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3
Q

vasculitis can affect any vessel

true or false?

A

true

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4
Q

Untreated small vessel vasculitis can have mortality rate of __% after 2 years

A

90%

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5
Q

what are the 2 different types of vasculitis classified as?

A

primary or secondary

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6
Q

what is primary vasculitis a result of?

A

no known cause - inflammatory response that targets the vessel walls.

Sometimes this is autoimmune.

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7
Q

what is secondary vasculitis triggered by?

A

infection
drug
toxin

or may occur as part of another inflammatory disorder or cancer.

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8
Q

name the the 2 causes of large-vessel vasculitis

A

takayasu arteritis

giant cell arteritis

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9
Q

name the 2 conditions under the umbrella term of medium-vessel vasculitis

A

polyarthritis nodosa

kawasaki disease

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10
Q

name the 2 different types of small vessel vasculitis

A
  1. ANCA-associated

2. non-ANCA associated/immune complex

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11
Q

list the 3 types of ANCA-associated small vessel vasculitis

A
  1. microscopic polyangitis
  2. Wegner - granulomatosis with polyangitis
  3. Churg-Strauss - eosinophilic granulomatosis with polyangitis
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12
Q

name 3 non-ANCA small-vessel vasculitis

A
  1. cryoglobulinemic vasculitis
  2. IgA vasculitis - Henoch-Schönlein
  3. hypocomplementemic urticarial vasculitis - Anti-C1q vasculitis
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13
Q

presentation of vasculitis is depend son what?

A

type of vessel affected

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14
Q

what are the systemic symptoms in vasculitis?

A

fever
malaise
weight loss
fatigue

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15
Q

takayasu arteritis is common in who? sex? age? ethnicity?

A

females
under 40 years old
asians

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16
Q

giant cell arteritis is common in who? age? what does it cause?

A

over 50 years old

causes temporal arteritis but aorta also common

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17
Q

what are the presenting features in large vessel vasculitis?

A

bruit in carotid artery
blood pressure
claudication

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18
Q

what is temporal arteritis associated with?

A

polymyalgia rheumatica

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19
Q

what is temporal arteritis?

A

unilateral headache
scalp tenderness
jaw claudication
temporal arteries prominent with reduced pulsation

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20
Q

what is there a risk of in temporal arteritis?

A

blindness due to ischaemia of optic nerve

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21
Q

what investigations do you carry out in GCA/temporal arteritis?

A

ESR
plasma viscosity
raised CRP
biopsy of artery

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22
Q

what is the management in GCA/temporal arteritis?

A

40-60mg prednisolone

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23
Q

what is the other name for granulomatosis with polyangitis (GPA)?

A

Wegener’s granulomatosis

24
Q

what is the other name for eosinophilic granulomatosis with polyangiitis (EGPA)?

A

Churg-Strauss syndrome

25
GPA is commoner in who? ethnicity? sex? age?
european descent males (only just) 35-55
26
common symptom in GPA is...
arthralgia
27
list the 4 points in the GPA classification criteria
1. nasal or oral inflammation 2. abnormal CXR 3. urinary sediment 4. granulomatous inflammation on biopsy 2 of these 4 to have GPA
28
list the ENT features in GPA
``` Sinusitis Nasal crusting Epistaxis Mouth ulcers Sensorineural deafness Otitis media and deafness “Saddle nose” due to cartilage ischaemia ```
29
list the respiratory manifestations in GPA
pulmonary infiltrates alveolar haemorrhage cavitating nodules on CXR
30
list the cutaneous manifestations in GPA
palpable purpura | cutaneous ulcers
31
name the renal condition in GPA
Necrotising glomerulonephritis
32
list the nervous system manifestations in GPA
Mononeuritis multiplex Sensorimotor polyneuropathy Cranial nerve palsies
33
name the ocular conditions in GPA
``` Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusion Proptosis ```
34
what is the main difference in EGPA compared to GPA?
late onset asthma and a high eosinophil count Many features of EGPA are similar to those of GPA
35
what is in the ACR criteria for EGPA?
Asthma (wheezing, expiratory rhonchi) Eosinophilia of more than 10% in peripheral blood Paranasal sinusitis Pulmonary infiltrates (may be transient) Histological proof of vasculitis with extravascular eosinophils Mononeuritis multiplex or polyneuropathy should have 4 or more
36
what investigation is used to detect ANCA?
immunofluoresence
37
name the 2 different types of ANCA
cANCA and pANCA
38
what condition is cANCA positive in?
GPA
39
what conditions is pANCA positive in?
MPO and EGPA
40
which ANCA is more circular on immunofluoresence?
cANCA
41
diagnose: cANCA 70-90% PR3 5-10% MPO
GPA
42
diagnose: pANCA <2% PR3 30-70% MPO
EGPA
43
diagnose: pANCA 10-30% PR3 70-90% MPO
MPA
44
cANCA and anti-PR3 are associated with...
GPA
45
pANCA and a low PR3 are associated with...
EGPA
46
pANCA and anti-MPO are associated with?
MPA
47
name the management in ANCA associated vasculitis (AAV): localised - generalised - refractory -
localised - MTX + steroids generalised - Cyclophosphamide + steroids followed by azathioprine refractory - IV Immunoglobulins Rituximab
48
diagnose: ``` 46 year old female hearing loss then deaf fatigue weight loss sinusitis rash over feet and legs right foot and left wrist drop clear chest ESR and CRP high normal renal mononeuritis multiplex cANCA and anti-PR3 positive ```
GPA/Wegener's granulomatosis
49
which Ig is Henoch-Schönlein purpura (HSP) mediated by?
acute immunoglobulin A (IgA)–mediated disorder
50
approximately 75% of cases of HSP occur in who?
children aged 2-11 years; HSP is rare in infants
51
more than 75% of patients have had preceding URTI, pharyngeal infection, or GI infection true or false?
true
52
what is the most common pathogen in Henoch-Schonlein Purpura (HSP)?
group A streptococcus
53
what is the presentation in HSP?
Purpuric rash typically over buttocks and lower limbs Colicky abdominal pain Bloody diarrhoea Joint pain +/- swelling Renal involvement (50%)
54
what is the management in HSP?
self-limiting tend to resolve in 8 weeks relapses possible urinalysis is essential
55
why does Peh Zhen Hui love gardening so much?
cause it gives him the chance to get down and dirty with his hoes