Vasculitis

Question 1

Small vessel vasculitis types

Answer 1

Henoch-Schonlein Purpura

Eosinophilic Granulomatosis with Polyangiitis

Microscopic polyangiitis

Granulomatosis with polyangiitis (Previously called Wegener’s granulomatosis)

Question 2

Medium vessel vasculitis types

Answer 2

Polyarteritis nodosa

Kawasaki Disease

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

Question 3

Large vessel vasculitis types

Answer 3

Giant cell arteritis

Takayasu’s arteritis

Question 4

Generic features of vasculitis that can occur

Answer 4

Purpura

Joint and muscle pain

Renal impairment and GN

GI disturbance e.g. diarrhoea, abdominal pain

Systemic features - fever, night sweats, weight loss etc…

Question 5

Investigations in suspected vasculitis

Answer 5

Presentation is quite vague so do rheumatology work up with:

• ANA
• ANCA
• RF
• CRP and ESR

FBC for eosinophilia seen in Eosinophilic Granulomatosis with Polyangiitis

Urine dip

Biopsy of affected organs is the gold standard for diagnosis e.g. kidneys in small vessel vasculitis, or blood vessels e.g. in GCA

Question 6

Eosinophilic granulomatosis with Polyangiitis presentation

Answer 6

Asthma or allergic symptoms
Eosinophilia

p-ANCA positive in 60%

Can cause other general features common to vasculitis:

• renal impairment, Rapidly progressing glomerulonephritis
• lung inflammation and haemoptysis
• Purpura

Question 7

Microscopic polyangiitis presentation

Answer 7

Main feature is renal failure - GN

Can also affect the lungs causing haemoptysis and SOB

Purpura

p-ANCA most commonly present (50-75%) but also sometimes c-ANCA (40%)

Question 8

Granulomatosis with polyangiitis presentation

Answer 8

Respiratory tract symptoms:

• Nasal discharge - crusty secretions
• Recurrent epistaxis/sinusitis
• Haemoptysis, dyspnoea
• Saddle shaped nose (due to perforated nasal septum)

Rapidly progressing GN also common (80% patients)

Purpura

cANCA positive in >90%
(pANCA in 25%)

Question 9

Management of vasculitis

Answer 9

Refer to specialist usually rheumatology

Management is with a combination of steroids and immunosuppressants

• Steroids can be oral, IV, nasal (for nasal Sx) or inhaled (for lung involvement e.g. in eosinophilic granulomatosis with Polyangiitis)
• Immunosuppressants can include cyclophosphamide, methotrexate and biologics

Question 10

What is polyarteritis nodosa? Presentation?

Answer 10

Medium vessel vasculitis

ANCA negative

Associated most with Hep B (but can occur without cause or with Hep C or HIV)

Presentation:

• Livedo reticularis
• Renal impairment
• Strokes and MI (causes thrombosis)

Question 11

What is Takayasu’s arteritis? Presentation?

Answer 11

Large vessel vasculitis

Mainly affects aorta and branches

These can form aneurysms

Presentation:

• Non-specific systemic symptoms e.g. fever, malaise, muscle aches
• Arm claudication
• Syncope

It is diagnosed with CT or MRI angiography