Vasculitis - GPA, EGPA, PAN, MPA, Henoch Schonlein Purpura

Question 1

Granulomatosis with polyangiitis
-presentation
-investigations
-management

Answer 1

ENT + RESP + KIDNEY
URT - nose bleeds, sinusitis
LRT - SOB, hemoptysis
Rapidly progressive glomerulonephritis => renal failure
Saddle nose
Vasculitic rash, eye involvement, cranial nerve lesion

CANCA
CXR - presentation varies
Renal biopsy - crescents in bowman’s capsule

CS
Cyclophosphamide
Plasma exchange

Question 2

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
-presentation
-investigations
-management

Answer 2

More allergic-like - asthma, eosinophilia
Sinusitis
Mononeuritis multiplex

PANCA
Eosinophilia

CS
Cyclophosphamide

Question 3

Polyarteritis nodosa
-presentation

Answer 3

Inflammation of vessel => intimal thickening and weakening => aneurysm => significant bleeding
-also causes organ infarcts, mainly kidney

Systemic - fever, malaise, weight loss, joint pain
CV - HTN
Neuro - mononeuritis multiplex, sensorimotor polyneuropathy
Renal - hematuria, renal failure, testicular pain
Derm - livedo reticularis
ASSOCIATION WITH HEP B

Biopsy of clinically affected organ to confirm
Blood - assess involvement of organs
CXR - rule out GPA
Imaging - arteriography or CT/MRI to assess for aneurysms

Mild with no end organ damage - PO pred
-alternative - azathioprine/methotrexate + tolerable pred dose

Moderate/severe with end organ damage - high dose GC + cyclophosphamide
-azathioprine/methotrexate for remission

Question 4

Microscopic polyangiitis
-presentation
-investigations
-management

Answer 4

Unknown trigger induces AI response against MPO and PR3 (PANCA, CANCA)

Systemic - fever, weight loss
MSK - myalgia, arthralgia
Neuro - peripheral neuropathy, convulsions, cerebral hemorrhage/infarct
Renal - rapidly progressive glomerulonephritis
Resp - pulmonary hemorrhage

Urine dip, protein:creatinine ratio - renal involvement => nephrology referral
Bloods
-FBC - exclude lymphoproliferative disorders
-LFT - systemic, liver involvement
-U&E - renal involvement
-CRP, ESR - can be high
-AB - ANA, RF, ANCA
Biopsy - depending on organ involved

Non severe - CS + methothrexate/mycophenolate mofetil
Severe - CS + cyclophosphamide
Refractory - vasculitis center referral + rituximab/IVIG

Maintenance - low dose CS + azathioprine

Question 5

PANCA
-associations with other conditions

Answer 5

UC
PSC
Anti GBM
Crohns

Question 6

Henoch Schonlein purpura
-presentation
-investigations
-management

Answer 6

IgA mediated small vessel vasculitis
Overlap with IgA nephropathy

IN CHILDREN AFTER INFECTION - TETRAD OF
-palpable purpuric rash over buttocks, ext surfaces
-abdo pain
-polyarthritis
-features of IgA nephropathy

No diagnostic tests but can rule out other differentials
-ITP
-SLE, RA
-IgA nephropathy

Self limiting
-simple analgesia - avoid NSAIDs in active GI bleeds
Renal involvement (declining renal function/nephrotic) - IV CS

Monitor
-6 months - periodic urine dip, BP to detect progressive renal disease