4: Vasculitis Flashcards
What is vasculitis?
What are the consequences of this?
Inflammation of blood vessels
Ischaemia and necrosis causing organ dysfunction
Vasculitis can affect ___ size of blood vessel but often presents in distinct ___.
any
patterns
Vasculitis tends to be a (single / multi-) system presentation.
multi-system
What tends to cause primary vasculitis?
Secondary vasculitis?
Primary - autoimmune
Secondary - infection, other stuff
What occurs in blood vessels in vasculitis?
Inflammation
Thickening of endothelium
Lumenal narrowing
Ischaemia / claudication…
Necrosis and organ damage
What are the two types of vasculitis you need to know about?
Large vessel vasculitis - Takayasu and Giant cell
Small vessel vasculitis - GwP (Wegeners) and eosinophilic (Churg-Strauss)
Which antibody is associated with the small vessel vasculitidies you need to know about?
ANCA
What are the constitutional symptoms often seen alongside organ damage in vasculitis?
Fever
Malaise
Weight loss
What are the two main types of large vessel vasculitis?
Giant cell arteritis
Takayasu arteritis
Takayasu arteritis tends to present in people of which age range?
What about giant cell arteritis?
< 40 years old
> 40 years old
large vessel vasculitis - takayasu:
bruit
claud
brachial brachial bp differences
affects aorta, subclav, femoral
revise GCA
associated with PMR
end artery to optic nerve
diagnosed by inflam markers and ta biopsy
What lights up on a PET CT scan?
Metabolically active cells
i.e those involved in inflammation
What sort of scan is useful in large vessel vasculitis?
PET CT
PET CT is good for visualising active inflammation - what is used to view structural changes?
CT / MRI
How is giant cell arteritis treated?
40-60mg prednisolone
How is Takayasu arteritis treated?
Steroid
Immunosuppressants
doesn’t burn out as quick as GCA
What percentage of people with GCA also have PMR?
40%ish
A higher dose of prednisolone is used in GCA if there is what?
Visual loss
What are the ANCA associated small vessel vasculitidies?
Wegener’s granulomatosis (now Granulomatosis with polyangiitis)
Churg-Strauss syndrome (now Eosinophilic granulomatosis with polyangiitis)
Microscopic polyangiitis (which affects the kidneys)
GPA is commoner in (men / women) of ___ descent.
men
European
What are non-specific features of GPA?
Constitutional symptoms
What are specific features of GWP?
ENT SYMPTOMS (nosebleeds, hearing loss)
CHEST SYMPTOMS
RASH
KIDNEY SYMPTOMS (also asymptomatic as in SLE, MUST be screened for, is the most important thing)
What test should be carried out immediately in someone with suspected GPA?
Urinalysis
What happens to the nose in someone with GPA?
Saddle nose
cartilage wears away
What is seen on CXR in someone with GWP?
Cavitating granulomas
What does a vasculitic rash look like?
Purpuric
Non-blanching
May ulcerate
What kind of kidney disease is seen in GPA?
Necrotising glomerulonephritis
What is a nervous manifestation of GPA?
Mononeuritis multiplex
Peripheral nerve stops working - sudden onset foot drop for example
What is an ocular manifestation of GWP?
Uveitis
Scleritis
What should make you suspect EGWP as opposed to GWP?
Late onset asthma
High eosinophil count
What are the key features which would make you suspect EGWP?
Asthma
Eosinophilia
Proof of vasculitis w/ eosinophils on biopsy
Are ENT symptoms more a feature of GWP or EGWP?
GWP
What does ANCA stand for?
Anti-neutrophil cytoplasmic antibody
What are the two types of ANCA?
cANCA
pANCA
What is cANCA associated with?
GWP
What is pANCA associated with?
EGWP
Microscopic polyangiitis
What is the antibody raised in active GWP?
PR3
What is the antibody raised in active EGPA?
MPO
so to recap
GPA - cANCA and PR3
EGPA - pANCA and MPO
When vasculitis is active, complement levels (increase / decrease).
decrease
as they are consumed
How is small vessel vasculitis treated?
IV steroid
IV cyclophosphamide
Vasculitis patients must be treated ___ and ___.
early
aggressively
Even if urinanalysis normal, what must be done in a new patient presenting with vasculitis?
Why?
Renal biopsy
Kidney involvement gives overall disease severity
Which disease is usually provoked by infection, self-limiting, and mediated by IgA?
Henoch-Schonlein purpura
What is the presentation of HSP?
Rash
+/- GI symptoms
+/- Cough
The majority of HSP cases are seen in ___.
children
What tends to cause HSP?
Group A Strep**. infection
Where is the rash of HSP seen?
Feet, legs, buttocks
What test should still be carried out in those with HSP?
Urinalysis
How long does HSP typically last?
2 months
Can HSP relapse?
Yes
