Vasculitis Flashcards
Dr J Golla
what are the core clinical problems seen in vasculitis?
joint pain/swelling abnormal bloods rash red/painful eye SOB cough heaache vision loss
define vasculitis
inflammation of blood vessels, often with ischemia, necrosis, and organ inflammation
vasculitis can affect any vessel
true or false?
true
Untreated small vessel vasculitis can have mortality rate of __% after 2 years
90%
what are the 2 different types of vasculitis classified as?
primary or secondary
what is primary vasculitis a result of?
no known cause - inflammatory response that targets the vessel walls.
Sometimes this is autoimmune.
what is secondary vasculitis triggered by?
infection
drug
toxin
or may occur as part of another inflammatory disorder or cancer.
name the the 2 causes of large-vessel vasculitis
takayasu arteritis
giant cell arteritis
name the 2 conditions under the umbrella term of medium-vessel vasculitis
polyarthritis nodosa
kawasaki disease
name the 2 different types of small vessel vasculitis
- ANCA-associated
2. non-ANCA associated/immune complex
list the 3 types of ANCA-associated small vessel vasculitis
- microscopic polyangitis
- Wegner - granulomatosis with polyangitis
- Churg-Strauss - eosinophilic granulomatosis with polyangitis
name 3 non-ANCA small-vessel vasculitis
- cryoglobulinemic vasculitis
- IgA vasculitis - Henoch-Schönlein
- hypocomplementemic urticarial vasculitis - Anti-C1q vasculitis
presentation of vasculitis is depend son what?
type of vessel affected
what are the systemic symptoms in vasculitis?
fever
malaise
weight loss
fatigue
takayasu arteritis is common in who? sex? age? ethnicity?
females
under 40 years old
asians
giant cell arteritis is common in who? age? what does it cause?
over 50 years old
causes temporal arteritis but aorta also common
what are the presenting features in large vessel vasculitis?
bruit in carotid artery
blood pressure
claudication
what is temporal arteritis associated with?
polymyalgia rheumatica
what is temporal arteritis?
unilateral headache
scalp tenderness
jaw claudication
temporal arteries prominent with reduced pulsation
what is there a risk of in temporal arteritis?
blindness due to ischaemia of optic nerve
what investigations do you carry out in GCA/temporal arteritis?
ESR
plasma viscosity
raised CRP
biopsy of artery
what is the management in GCA/temporal arteritis?
40-60mg prednisolone
what is the other name for granulomatosis with polyangitis (GPA)?
Wegener’s granulomatosis
what is the other name for eosinophilic granulomatosis with polyangiitis (EGPA)?
Churg-Strauss syndrome
GPA is commoner in who? ethnicity? sex? age?
european descent
males (only just)
35-55
common symptom in GPA is…
arthralgia
list the 4 points in the GPA classification criteria
- nasal or oral inflammation
- abnormal CXR
- urinary sediment
- granulomatous inflammation on biopsy
2 of these 4 to have GPA
list the ENT features in GPA
Sinusitis Nasal crusting Epistaxis Mouth ulcers Sensorineural deafness Otitis media and deafness “Saddle nose” due to cartilage ischaemia
list the respiratory manifestations in GPA
pulmonary infiltrates
alveolar haemorrhage
cavitating nodules on CXR
list the cutaneous manifestations in GPA
palpable purpura
cutaneous ulcers
name the renal condition in GPA
Necrotising glomerulonephritis
list the nervous system manifestations in GPA
Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies
name the ocular conditions in GPA
Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusion Proptosis
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
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
what investigation is used to detect ANCA?
immunofluoresence
name the 2 different types of ANCA
cANCA and pANCA
what condition is cANCA positive in?
GPA
what conditions is pANCA positive in?
MPO and EGPA
which ANCA is more circular on immunofluoresence?
cANCA
diagnose:
cANCA
70-90% PR3
5-10% MPO
GPA
diagnose:
pANCA
<2% PR3
30-70% MPO
EGPA
diagnose:
pANCA
10-30% PR3
70-90% MPO
MPA
cANCA and anti-PR3 are associated with…
GPA
pANCA and a low PR3 are associated with…
EGPA
pANCA and anti-MPO are associated with?
MPA
name the management in ANCA associated vasculitis (AAV):
localised -
generalised -
refractory -
localised - MTX + steroids
generalised - Cyclophosphamide + steroids
followed by azathioprine
refractory - IV Immunoglobulins
Rituximab
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
which Ig is Henoch-Schönlein purpura (HSP) mediated by?
acute immunoglobulin A (IgA)–mediated disorder
approximately 75% of cases of HSP occur in who?
children aged 2-11 years; HSP is rare in infants
more than 75% of patients have had preceding URTI, pharyngeal infection, or GI infection
true or false?
true
what is the most common pathogen in Henoch-Schonlein Purpura (HSP)?
group A streptococcus
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%)
what is the management in HSP?
self-limiting
tend to resolve in 8 weeks
relapses possible
urinalysis is essential
why does Peh Zhen Hui love gardening so much?
cause it gives him the chance to get down and dirty with his hoes
