Small vessel vasculitis

Question 1

What is small vessel vasculitis

Answer 1

Necrotising polyangiitis that affects capillaries, venules, and arterioles

Question 2

When does small vessel vasculitis usually present?

Answer 2

5th - 7th decade

Question 3

What are the 3 main types of small vessel vasculitis?

Answer 3

Granulomatosis with polyangiitis (GPA)
Microscopic polyangiitis (MPA)
Eosinophilic GPA (EGPA)

Question 4

Characteristics of GPA?

Answer 4

• Predominantly anti-PR3 (cANCA) antibodies
• Necrotising granulomatous inflammation
• Lung involvement (pulmonary/renal syndrome)

Question 5

Characteristics of MPA?

Answer 5

• Predominantly anti-MPO (pANCA) antibodies
• Small vessel vasculitis with no granulomas
• Systemic features, renal, lung, skin, GI, nerves

Question 6

Characteristics of EGPA?

Answer 6

• Associated with asthma and eosinophilia
• 2/3 have skin involvement, generally no kidney involvement

Question 7

What are some constitutional symptoms of small vessel vasculitis?

Answer 7

fever, migratory arthralgia, weight loss, anorexia, malaise

Question 8

What investigations are required in small vessel vasculitis?

Answer 8

Urinalysis - Blood and protein
Bloods - Raised inf. markers, AKI, anaemia
Immunology - ANCA
Renal biopsy - Crescentic, pauci-immune

Question 9

Which ANCA is predominant in GPA?

Answer 9

cANCA - Anti-PR3

Question 10

Which ANCA is predominant in MPA?

Answer 10

pANCA - Anti-MPO

Question 11

How is small vessel vasculitis managed?

Answer 11

• Immunosuppression - steroids, cyclophosphamide/rituximab
• Plasma exchange
• Supportive - dialysis, ventilation