Vasculitis

Question 1

Give examples of large vessel, medium vessel and small vessel vasculitities?

Answer 1

Large vessel - takayasu vasculitis, giant cell arteritis

Medium vessel - polarteritis nodosa, Kawasaki disease

Small vessel - ANCA associated (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) and immune mediated (Cryoglobulinaemic vasculitis, IgA vasculitis)

Question 2

What is the other name for eosinophilic granulomatosis with polyangiitis?

Answer 2

Churg-Strauss syndrome

Question 3

How can takayasu’s vasculitis present?

Answer 3

With stenosis, occlusion or aneurysm of the large vessels (particularly the aorta)
There may be aortic regurgitation, vascular bruit and unequal BP measurement in different arms

Question 4

In what demographic does takayasu’s vasculitis typically present?

Answer 4

Women <40 years

Question 5

How is Takayasu’s vasculitis managed?

Answer 5

Corticosteroids

Question 6

In what demographic does Kawasaki’s disease typically present?

Answer 6

Young children

Question 7

How can Kawasaki’s disease present?

Answer 7

Fever
Polymorphic rash
Conjunctivities
Strawberry tongue
Cervical lymphadenopathy
Heart disease

Question 8

How is Kawasaki’s disease managed?

Answer 8

IVIG and/or aspirin

Question 9

Which virus is associated with polyarteritis nodosa?

Answer 9

Hepatitis B and C

Question 10

What is the classic sign of polyarteritis nodosa on angiography?

Answer 10

Rosary sign (presence of multiple microaneurysms)

Question 11

How is polyarteritis nodosa managed?

Answer 11

Corticosteroids

Cyclophosphamide

Question 12

How can polarteritis nodosa present?

Answer 12

• Nervous system (in 55% of patients)
  
  • Mononeuritis multiplex, encephalopathy, seizures, stroke
• Skin (in 44%)
  
  • Purpura, nodules, Livedo reticularis, ulcers, bullous or vesicular eruptions, segmental skin oedema
• Abdominal organs (in 33%)
  
  • Abdominal pain, liver/bowel/spleen infarction, bowel perforation, bleeding
• Kidneys (in 11%)
  
  • Renal impairment, renal infarcts
• Musculoskeletal system (in 24% to 80%).
  
  • Arthritis, arthralgia, myalgia, muscle weakness

Question 13

What conditions is type 1 cryoglobulinaemic vasculitis associated with?

Answer 13

Haematological malignancy

Question 14

What condition is type 2 cryoglobulinaemic vasculitis associated with?

Answer 14

Hepatitis C

Question 15

How is cryoglobulinaemic vasculitis managed?

Answer 15

Treat the underlying condition (haem malignancy in type 1 and hep C in type 2)

Immunosuppressants in type 2 disease

Question 16

How should vasculitis be investigated?

Answer 16

CRP / ESR - elevated
ANCA - positive in ANCA associated small vessel vasculitities
U+Es, urinalysis +/- kidney biopsy - to investigate renal involvement
Other biopsy (skin, vessel) as appropriate

Question 17

What is the gold standard diagnostic test for takayasu arteritis?

Answer 17

Angiography