Small to Medium Vessel Vasculitis

Question 1

how does it present?

Answer 1

fever and weight loss 
raised, non-blanching purpuric rash 
arthralgia/ arthritis 
mononeuritis multiplex 
glomerulonephritis 
lung opacities on x ray

Question 2

how are they classified?

Answer 2

ANCA associated (AAV): Wegener’s, Microscopic polyangiitis, Churg-Strauss Syndrome
o No granuloma: Microscopic polyangiitis (MPA)
o Granuloma
- No asthma & Eosinopgilia: Wegener’s granulomatosis = Granulomatosis with polyangiitis (GPA)
- Asthma & Eosinophilia: Churg-Strauss Syndrome = Eosinophilic granulomatosis with polyangiitis (EGPA)
Non-ANCA associated: Henoch-Schnlein purpura, Serum cryoglobulin?
o Not IgA demoinant immune deposit:
- No serum cryoglobulin: Other non ANCA vasculitis (e.g. IBD vasculitis)
- Serum cryoglobulin: Cyroglobulinemia
o IgA dominant immune deposit: Henoch-Schnlein purpura

Question 3

another name for Wegener’s granulomatosis

Answer 3

granulomatosis with polyangiitis (GPA)

Question 4

another name for Churg-Strauss syndrome

Answer 4

Eosinophilic granulomatosis with polyangiitis (EGPA)

Question 5

another name for microscopic polyangiitis (MPA)

Answer 5

microscopic polyangiitis

Question 6

what are ANCAs

Answer 6

Anti-neutrophil cytoplasmic antibodies (ANCAs)
- they are autoantibodies against antigens in the cytoplasm of neutrophil granulocytes
immunofluorescence can detect ANCA and differentiate ANCA patterns

Question 7

what is the ANCA for GPA

Answer 7

cANCA

Question 8

what is the ANCA for EGPA

Answer 8

pANCA

Question 9

what is the ANCA for MPA

Answer 9

pANCA