Vasculitis

Question 1

What are the large vessel vasculitides?

Answer 1

Giant cell arteritis (GCA)

Takayasu arteritis

Question 2

What is giant cell arteritis (GCA)?

Answer 2

Large vessel vasculitis causing skip lesions of granulomatous inflammation causing vessel wall thickening narrowing the lumen and causing distal ischaemia

Question 3

How does giant cell arteritis (GCA) present?

Answer 3

Over 50s

Headache

Scalp tenderness

Prominent, tender temporal artery

Painless visual disturbances

Jaw claudication

Question 4

What condition is giant cell arteritis (GCA) associated with and how does it present?

Answer 4

Polymyalgia rheumatica

Proximal muscle pain and stiffness

Question 5

What blood test would you order in suspected giant cell arteritis (GCA) and what would it show?

Answer 5

Inflammatory markers

Raised

Question 6

What is the diagnostic test for giant cell arteritis (GCA)? What do you do if it is negative but there is a strong clinical suspicion of GCA?

Answer 6

Temporal artery biopsy showing granulomatous inflammation

Treat as giant cell arteritis (GCA). Biopsy has high false negative rate due to skip lesion pattern

Question 7

How is giant cell arteritis (GCA) treated?

Answer 7

Immediate prednisolone

• 40mg
• 60mg if visual symptoms

Question 8

What can untreated giant cell arteritis (GCA) progress to and why?

Answer 8

Irreversible blindness from optic nerve ischaemia

Question 9

How does Takayasu arteritis present?

Answer 9

Vasculitis in Asian females under 40

Question 10

What are the ANCA-positive small vessel vasculitides?

Answer 10

Granulomatosis with polyangiitis (GPA)

Eosinophilic granulomatosis with polyangiitis (EGPA)

Microscopic polyangiitis

Question 11

What are the ANCA-negative small vessel vasculitides?

Answer 11

Henoch-Schloein purpura (HSP)

Question 12

What is granulomatosis with polyangiitis (GPA)?

Answer 12

Small vessel ANCA associated vasculitis causing ENT symptoms and kidney damage

Question 13

What immunological markers are associated with granulomatosis with polyangiitis (GPA)

Answer 13

c-ANCA

PR3

Question 14

How does granulomatosis with polyangiitis (GPA) present?

Answer 14

Constitutional symptoms

ENT symptoms (saddle nose, epistaxis, sinusitis, heating loss etc.)

Cough (haemoptysis)

Vasculitic rash

Mononeuritis multiplex

Question 15

How does c-ANCA appear under immunofluorsence

Answer 15

Cytoplasmic-anti-neutrophil-cytoplasmic antibody

Stains around the cytoplasm

Question 16

What is eosinophilic granulomatosis with polyangiitis (EGPA)?

Answer 16

Small vessel vasculitis causing asthma and eosinophilia

Question 17

How does eosinophilic granulomatosis with polyangiitis (EGPA) present?

Answer 17

Constitutional symptoms

Asthma

Vasculitic rash

Mononeuritis multiplex

Question 18

What immunological markers are associated with eosinophilic granulomatosis with polyangiitis (EGPA)?

Answer 18

p-ANCA

MPO

Question 19

How are the ANCA-associated small vessel vasculitides treated?

Answer 19

Depends on severity

Just respiratory involvement
- methotrexate and steroids

Organ dysfunction/failure

• cyclophosphamide and steroids
• and plasma exchange if creatinine >50
• then further DMARDs

Refractory
- IV immunoglobulins

Question 20

What is Henoch-Schonein purpura (HSP)?

Answer 20

IgA mediated small vessel vasculitis commonly affecting children

Question 21

How does Henoch-Schonein purpura (HSP) present?

Answer 21

In children

Constitutional symptoms

Abdominal pain and bloody diarrhoea

Sore throat/cough

Arthralgia

Question 22

What test needs to be done in suspected small vessel vasculitis and what are you looking for in this test?

Answer 22

Urinalysis

Proteinuria/haematuria which suggests kidney damage

Question 23

How is Henoch-Schonein purpura (HSP) treated?

Answer 23

Self limiting condition so no management required

May relapse