Small Cell Vasculitis Flashcards

1
Q

what is this divided into

A

ANCA associated vasculitis (AAV) and ANCA negative

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2
Q

what are the different kinds of ANCA associated (AAV)

A

granulomatosis with polyangiitis (GPA - previously Wegeners)
microscopic polyangiitis (MPA)
renal limited vasculitis (RLV)
eosinophilic granulomatosis with polyangiitis (EGPA - previously churg-strauss)

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3
Q

what is the kind of ANCA negative

A

henoch-schonlein purpura (HSP)

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4
Q

what is prevalence of GPA (AAV)

A

northern european descent
male:female - 1.5:1
age = 35-55 years

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5
Q

what are the ENT symptoms of GPA (AAV)

A
sinusitis 
nasal crusting 
epistaxis
mouth ulcers
sensorineural deafness
otitis media and deafness
saddle nose due to cartilage ischaemia
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6
Q

what are respiratory symptoms of GPA (AAV)

A
pulmonary infiltrates 
cough 
haemoptysis 
diffuse alveolar haemorrhage 
cavitating nodules on CXR
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7
Q

what are cutaneous symptoms of GPA (AAV)

A

palpable purpura

ulcers

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8
Q

what are renal symptoms of GPA (AAV)

A

necrotising glomerulonephritis

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9
Q

what are NS symptoms of GPA (AAV)

A

mononeuritis multiplex
sensorimotor polyneuropathy
cranial nerve palsies

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10
Q

what are ocular symptoms of GPA (AAV)

A
conjunctivitis 
episcleritis 
uveitis 
optic nerve vasculitis 
renal artery occlusion 
proptosis
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11
Q

what is symptoms of EGPA (AAV)

A

many similar to those of GPA but this has late onset asthma and high eosinophil count

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12
Q

how is AAV diagnosed

A

immunofluorescence:
cANCA = GPA
pANCA = MPA and EGPA

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13
Q

what is treatment of localised (upper or lower resp tract disease with no systemic involvement) or early (any, without organ or life threatening) AAV

A

methotrexate (or azathioprine) + steroids

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14
Q

what is treatment of generalised or systemic AAV (renal or other organ failure)

A

cyclophosphamide + steroids (1st line)
rituximab + steroids (alternative)
plasma exchange if creatine >500 followed by azathioprine

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15
Q

what is treatment of refractory AAV (progressive disease unresponsive to steroids and cyclo)

A

IV immunoglobulins

Rituximab

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16
Q

what is prognosis of AAV

A

untreated = fatal
treated = 5 year survival 90%
mortality in treated patients = 2.6x higher

17
Q

what is henoch-schonlein purpura (HSP)

A

acute IgA mediated disorder of general vasculitis involving smell vessels of skin, GI tract, kidneys, joints and rarely lungs and CNS

18
Q

what age does HSP occur

A

approx 75% occur in children 2-11; HSP is rare in infants

19
Q

what causes HSP

A

> 75% have preceding URTI, pharyngeal infection or GI infection 1-3 weeks before
group A strep!

20
Q

what are symptoms of HSP

A
purpuric rash over buttocks and lower limbs
cockily abdominal pain 
bloody diarrhoea 
joint pain +/- swelling 
renal involvement (50%)
21
Q

how is HSP treated

A

usually self limiting

tends to resolve in 8 weeks but relapses may occur

22
Q

how is HSP followed up

A

essential to perform urinalysis to screen for renal involvement