Vasculitis Flashcards
Which vasculitis have granulomas? (4)
GPA
EPG
Takayasu
GCA
What are the small vessel vasculitites?
Wegner's Churg -Strauss Microscopic polyangitis HSP Hypersensitivity vasculitis Vasculitis secondary to CTS Vasculitis secondary to viral infection Cryoglobulinaemic Lupus vasculitis Behcet's Goodpasture's Paraneoplastic
What are the medium vessel vasculitis?
Polyarteritis nodosa
Kawasaki
Isolate CNS vasculitis
What are the large vessel vasculitis?
GCA
Takayasu
Which vasculitis do you get ANCA in?
Wegner’s, MPA, Churg strauss, drug induced, IBD, rheumatoid vasculitis and other CTD associated vasc
PR3 in CF patients
pANCA correlates with
cANCA correlates with
pANCA- MPO
cANCA-PR3
MPO associated with?
MPA 70%
Wegner’s 10%- haematuria and lower relapse rate
Anti-GBM (40%)
PR3 associated with?
Wegner’s - higher relapse rate, more ENT disease
Clinical features when suspect a vasculitis?
Palpable purpura Nose bleeds, sinusitis Fever Haemoptysis Mononeuritis multiplex Fatigue, weakness, arthralgia Abdominal pain Hypertension and renal insuffieciency
What does finding of “atypical” ANCA suggest?
Other than vasculitis eg CTD, IBD, autoimmune hepatitis
What proportion of Churg Strauss can be ANCA associated?/
50%
Can be MPO or PR3
What do you expect on renal limited vasculitis biopsy?
Little or no deposition of immune reactants like Ig and complement proteins.
Classic vasculitis drugs for ANCA positive?
Hydralazine Phenytoin PTU allopurinol sulfasalazine minocycline cipro clozapine
What is the ANCA positivity rates for UC and Crohn’s?
60-80% UC and PSC
10-25% Crohn’s
Do GPA, CSS, MPA have immune deposits?
Unlike other causes small vessel vasculitis- no
best way to distinguish churg strauss from MPA
asthma and eosinophilia
Do you HAVE to get a biopsy?
YES!
For god’s sake the toxicity of treatment is too high to get it wrong.
Need a biopsy ?pauci-immune crescentic GN
But don’t wait for results, get
True or false- S aureus in the nose increases risk Wegner’s?
False
Increases risk relapse but no evidence that it is pathogenic.
What test should everyone with Wegner’s have done?
Alpha 1 AT genotype
not levels- as is an acute phase reactant
Classic Wegner’s symptoms?
Upper: Nasal or oral inflammation with bloody discharge, OM, subglottic stenosis
Chest: cough, wheeze, fleeting pulm infiltrates
Migratory arthralgias
Microscopica haematuria
Conjuntivitis, episcleritis, proptosis, diplopia, uveitis
heart: conduction defects, pericarditis, coronary vasculopathy
Histopath of Wegner’s
pauci-immune crescentic GN or focal and segmental
