Small Cell Vasculitis Flashcards
what is this divided into
ANCA associated vasculitis (AAV) and ANCA negative
what are the different kinds of ANCA associated (AAV)
granulomatosis with polyangiitis (GPA - previously Wegeners)
microscopic polyangiitis (MPA)
renal limited vasculitis (RLV)
eosinophilic granulomatosis with polyangiitis (EGPA - previously churg-strauss)
what is the kind of ANCA negative
henoch-schonlein purpura (HSP)
what is prevalence of GPA (AAV)
northern european descent
male:female - 1.5:1
age = 35-55 years
what are the ENT symptoms of GPA (AAV)
sinusitis nasal crusting epistaxis mouth ulcers sensorineural deafness otitis media and deafness saddle nose due to cartilage ischaemia
what are respiratory symptoms of GPA (AAV)
pulmonary infiltrates cough haemoptysis diffuse alveolar haemorrhage cavitating nodules on CXR
what are cutaneous symptoms of GPA (AAV)
palpable purpura
ulcers
what are renal symptoms of GPA (AAV)
necrotising glomerulonephritis
what are NS symptoms of GPA (AAV)
mononeuritis multiplex
sensorimotor polyneuropathy
cranial nerve palsies
what are ocular symptoms of GPA (AAV)
conjunctivitis episcleritis uveitis optic nerve vasculitis renal artery occlusion proptosis
what is symptoms of EGPA (AAV)
many similar to those of GPA but this has late onset asthma and high eosinophil count
how is AAV diagnosed
immunofluorescence:
cANCA = GPA
pANCA = MPA and EGPA
what is treatment of localised (upper or lower resp tract disease with no systemic involvement) or early (any, without organ or life threatening) AAV
methotrexate (or azathioprine) + steroids
what is treatment of generalised or systemic AAV (renal or other organ failure)
cyclophosphamide + steroids (1st line)
rituximab + steroids (alternative)
plasma exchange if creatine >500 followed by azathioprine
what is treatment of refractory AAV (progressive disease unresponsive to steroids and cyclo)
IV immunoglobulins
Rituximab
what is prognosis of AAV
untreated = fatal
treated = 5 year survival 90%
mortality in treated patients = 2.6x higher
what is henoch-schonlein purpura (HSP)
acute IgA mediated disorder of general vasculitis involving smell vessels of skin, GI tract, kidneys, joints and rarely lungs and CNS
what age does HSP occur
approx 75% occur in children 2-11; HSP is rare in infants
what causes HSP
> 75% have preceding URTI, pharyngeal infection or GI infection 1-3 weeks before
group A strep!
what are symptoms of HSP
purpuric rash over buttocks and lower limbs cockily abdominal pain bloody diarrhoea joint pain +/- swelling renal involvement (50%)
how is HSP treated
usually self limiting
tends to resolve in 8 weeks but relapses may occur
how is HSP followed up
essential to perform urinalysis to screen for renal involvement
