Small Cell Vasculitis

Question 1

what is this divided into

Answer 1

ANCA associated vasculitis (AAV) and ANCA negative

Question 2

what are the different kinds of ANCA associated (AAV)

Answer 2

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

Question 3

what is the kind of ANCA negative

Answer 3

henoch-schonlein purpura (HSP)

Question 4

what is prevalence of GPA (AAV)

Answer 4

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

Question 5

what are the ENT symptoms of GPA (AAV)

Answer 5

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

Question 6

what are respiratory symptoms of GPA (AAV)

Answer 6

pulmonary infiltrates 
cough 
haemoptysis 
diffuse alveolar haemorrhage 
cavitating nodules on CXR

Question 7

what are cutaneous symptoms of GPA (AAV)

Answer 7

palpable purpura

ulcers

Question 8

what are renal symptoms of GPA (AAV)

Answer 8

necrotising glomerulonephritis

Question 9

what are NS symptoms of GPA (AAV)

Answer 9

mononeuritis multiplex
sensorimotor polyneuropathy
cranial nerve palsies

Question 10

what are ocular symptoms of GPA (AAV)

Answer 10

conjunctivitis 
episcleritis 
uveitis 
optic nerve vasculitis 
renal artery occlusion 
proptosis

Question 11

what is symptoms of EGPA (AAV)

Answer 11

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

Question 12

how is AAV diagnosed

Answer 12

immunofluorescence:
cANCA = GPA
pANCA = MPA and EGPA

Question 13

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

Answer 13

methotrexate (or azathioprine) + steroids

Question 14

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

Answer 14

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

Question 15

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

Answer 15

IV immunoglobulins

Rituximab

Question 16

what is prognosis of AAV

Answer 16

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

Question 17

what is henoch-schonlein purpura (HSP)

Answer 17

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

Question 18

what age does HSP occur

Answer 18

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

Question 19

what causes HSP

Answer 19

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

Question 20

what are symptoms of HSP

Answer 20

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

Question 21

how is HSP treated

Answer 21

usually self limiting

tends to resolve in 8 weeks but relapses may occur

Question 22

how is HSP followed up

Answer 22

essential to perform urinalysis to screen for renal involvement