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CNSLF Pathology - Vasculitis (ii) (Bacterial endocarditis (dx…
CNSLF Pathology - Vasculitis (ii)
Bacterial endocarditis
infection so DON'T TX WITH IMMUNOSUPPRESSION - if you misdx this as a primary vasculitis the immunosuppression will cause sepsis
dx
transthoracic/transoesophageal ECHO - vegetations
clinical suspicion - PUO
blood cultures
acute phase response
immunological stim leads to additional tissue destruction
immune complex-mediated tissue damage
acute: infection dominates clinical picture - esp in IVDU
subacute: immunological features may dominate
vegetations = friable masses composed of inflamm components (e.g. fibrin) + microorganisms - septic emboli risk
symptoms + signs due to...
damage to valves
ICD
systemic illness a/w widespread inflamm
tx = antibiotics
txing infection removes antigens
immune complexes no longer formed
v rarely needs steroids
Cryoglobulinaemia
Igs which precipitate in the cold
clinical manifestations due to...
Raynaud's phenomenon
reduced blood flow esp to fingers + feet due to art spasm
can compromise a digit (pregangrene)
numbness (mononeuritis multiplex - reduced blood flow to N)
vasculitis (anywhere)
joint
renal
neuropathy
GI involvement
causes palpable (raised) purpura (not to be confused with flat purport which are due to clothing defects
Meltzer's triad
purpura
arthralgia
weakness
testing cryoglobulins
serum taken + kept @ 37 degrees til separated in lab
1ml serum drain into 1ml syringe (preheat the tubes)
keep @ 4 degrees for 72-168hrs (3-7 days)
if precipitate there - estimate %
check that cryo goes back in solution when rewarmed to 37 degrees - if it won't its fibrin
when +ve (will be cloudy) type + assess for causes
causes of false -ves
syringe wasn't warmed to 37 degrees
sample not kept @ 37 degrees until clotting is completed
sample is centrifuged @ temps below 37 degrees
the sample isn't stored @ 4 degrees for @ least 72hrs
types
I = monoclonal
M band +ve
must classify M band for tx (use electrophoresis)
G
A
M
kappa
lambda
myeloma
Waldenstrom's macroglobulinaemia
B cell leukaemias/lymphomas
II = mixed
monoclonal component + polyclonal Igs
most common
HBV, HCV
CTD
B cell lymphoprolif disease
mixed essential (idiopathic) cryoglobulinaemia
III = polyclonal
immune complexes
SBE
CTD e.g. SLE
chronic infection
Tx
underlying causes
steroids +/- immunosuppression
plasmapheresis if life/organ threatening
Qs to ask
could this be CTD/vasculitis?
What organs may be involved?
Does it fit a classical pattern?
is serology helpful?
what site for bx/angiogram?
Potentially helpful tests
RF, anti-CCP
ANF, anti-dsDNA, anti-ENA
ANCA - anti-PR3, anti-MPO
anti-cardiolipin, anti-b2-gp1, lupus anticoagulant
complement
cryoglobulins
bx
angiography
Assess disease extent
clinical evaluation
FBC + abs if cytopenias
x-ray joints - involved + typical
CXR, PFTs, high res CT
U+E, urinalysis + microscopy, GFR, 24hr collection
CK, EMG, muscle bx
ECG, ECHO, Holter (if patient has palpitations), enzymes
neuroimaging + neurophysiology
Assess disease activity
inflamm
organ function
immunology