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Musculoskeletal > Vasculitis > Flashcards

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
Q

GPA is commoner in who? ethnicity? sex? age?

A

european descent
males (only just)
35-55

26
Q

common symptom in GPA is…

A

arthralgia

27
Q

list the 4 points in the GPA classification criteria

A
  1. nasal or oral inflammation
  2. abnormal CXR
  3. urinary sediment
  4. granulomatous inflammation on biopsy

2 of these 4 to have GPA

28
Q

list the ENT features in GPA

A 
Sinusitis
Nasal crusting
Epistaxis
Mouth ulcers
Sensorineural deafness
Otitis media and deafness
“Saddle nose” due to cartilage ischaemia
29
Q

list the respiratory manifestations in GPA

A

pulmonary infiltrates
alveolar haemorrhage
cavitating nodules on CXR

30
Q

list the cutaneous manifestations in GPA

A

palpable purpura

cutaneous ulcers

31
Q

name the renal condition in GPA

A

Necrotising glomerulonephritis

32
Q

list the nervous system manifestations in GPA

A

Mononeuritis multiplex

Sensorimotor polyneuropathy

Cranial nerve palsies

33
Q

name the ocular conditions in GPA

A 
Conjunctivitis 
Episcleritis 
Uveitis 
Optic nerve vasculitis 
Retinal artery occlusion  
Proptosis
34
Q

what is the main difference in EGPA compared to GPA?

A

late onset asthma and a high eosinophil count

Many features of EGPA are similar to those of GPA

35
Q

what is in the ACR criteria for EGPA?

A

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
Q

what investigation is used to detect ANCA?

A

immunofluoresence

37
Q

name the 2 different types of ANCA

A

cANCA and pANCA

38
Q

what condition is cANCA positive in?

A

GPA

39
Q

what conditions is pANCA positive in?

A

MPO and EGPA

40
Q

which ANCA is more circular on immunofluoresence?

A

cANCA

41
Q

diagnose:

cANCA
70-90% PR3
5-10% MPO

A

GPA

42
Q

diagnose:

pANCA
<2% PR3
30-70% MPO

A

EGPA

43
Q

diagnose:

pANCA
10-30% PR3
70-90% MPO

A

MPA

44
Q

cANCA and anti-PR3 are associated with…

A

GPA

45
Q

pANCA and a low PR3 are associated with…

A

EGPA

46
Q

pANCA and anti-MPO are associated with?

A

MPA

47
Q

name the management in ANCA associated vasculitis (AAV):

localised -
generalised -
refractory -

A

localised - MTX + steroids

generalised - Cyclophosphamide + steroids
followed by azathioprine

refractory - IV Immunoglobulins
Rituximab

48
Q

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
A

GPA/Wegener’s granulomatosis

49
Q

which Ig is Henoch-Schönlein purpura (HSP) mediated by?

A

acute immunoglobulin A (IgA)–mediated disorder

50
Q

approximately 75% of cases of HSP occur in who?

A

children aged 2-11 years; HSP is rare in infants

51
Q

more than 75% of patients have had preceding URTI, pharyngeal infection, or GI infection

true or false?

A

true

52
Q

what is the most common pathogen in Henoch-Schonlein Purpura (HSP)?

A

group A streptococcus

53
Q

what is the presentation in HSP?

A

Purpuric rash typically over buttocks and lower limbs

Colicky abdominal pain

Bloody diarrhoea

Joint pain +/- swelling

Renal involvement (50%)

54
Q

what is the management in HSP?

A

self-limiting
tend to resolve in 8 weeks
relapses possible
urinalysis is essential

55
Q

why does Peh Zhen Hui love gardening so much?

A

cause it gives him the chance to get down and dirty with his hoes

Musculoskeletal (37 decks)

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