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CNSLF Pathology - Vasculitis (i) (Secondary (malignancy/neoplasia (can…
CNSLF Pathology - Vasculitis (i)
Intro
systemic disease characterised by inflamm within blood vessels disrupting the int elastic lamina
changes from antithrombotic to pro
comp = thrombosis
lymphocytes in wall
fibrinoid necrosis, no nuclei, due to ICD
may involve 1 or many organs
may involve vessels of 1 size or main vessel types
2 cases of the same vasculitis can have v different presentations
minor presentations (e.g. localised nasal disease) can still reduce QOL (e.g. lots of nasal toilette)
Clinical features
ischaemia of tissues supplied by damaged vessels
features of widespread inflamm
myalgia
fever
fatigue
anorexia
weightloss (as inflamm = catabolic)
elevated CRP/ESR
vessel rupture extremely rare
Primary
multisystem diseases of unknown aetiology - tx similar for all
some have granulomata but can be missed on bx (sampling error)
classified based on size of vessel predominantly involved
large vessel
temporal arteritis
has granulomata
usually in older people
sometimes nodules in art palpable
risk of blindness if untxed
v responsive to steroids
bx to prove dx
medium vessel
granulomata
eosinophilic granulomatosis with polyangitis (EGPA)
formerly known as Churg-strauss syndrome
often a/w prodrome incl adult onset hard to tx asthma
no granulomata
PAD
a/w aneurysms - look for them on angiogram
coeliac vessels commonly affected
bx not always possible for dx
Kawasaki syndrome
due to superantigen
increased T cells overwhelm regulatory mechanisms
common in children
small vessel
granulomata
GPA (formerly known as Wegener's granulomatosis)
no granulomata
microscopic polyangitis (affects lungs + kidney)
Henoch Schonlein Purpura
generally benign
common in children
vasculitis type rash on lower limbs
GI + renal involvement
clinical features highly variable with each syndrome
morbidity + mortality due to...
organ failure/dysfunction - renal, pul, neuro
effects of immunosuppression
infections
sepsis
malignancy e.g. SCC when is more invasive in immunosuppressed than normally
cyclophosphamide causes infertility in females
Secondary
i.e. vessel inflamm due an underlying cause
infection (e.g. SBE)
drugs
malignancy/neoplasia
can mimic each other (e.g. weightloss, fatigue)
CF (10% of CF patients get vasculitis @ some point)
radiation
don't send bx from radiation field as post radiation changes difficult to differentiate from vasculitis
cryoglobulinaemia
CTD
Dx
serology - ANCA usually +ve
bx affected organ (occassionally blind bx - if so then go for muscle)
angiography - large vessel disease
GPA
multisystem disease of unknown aetiology
clinicopathological syndrome
most patients with active systemic disease are ANCA +ve
relapsing, remitting course - lifelong follow-up required
clinical features in...
upper/lower resp tract
kidneys (GN)
any organ
lab features
granulomata
vasculitis
necrosis
ANCA +ve
ANCA
cANCA
most anti-PR3 (IgG)
rarely a false +ve
closely a/w vasculitis
nuclei don't stain - stay dark
pANCA
50% anti-MPO (seen in IBD + other inflamm conditions)
less specific
50-70% ANCA +ve patients have vasculitis
false +ves in
infection
inflamm diseases
other, incl lymphoma
can be used for disease monitoring
raised ANCA has 70% PPV for a flare
when MPO or PR3 increase, assess for flare/relapse (can be as bad as 1st presentation)
if no clinical evidence for a flare monitor closely
can tx with plasmapheresis to remove Abs