23. ZTF vasculitis

Question 1

what is vasculitis

Answer 1

the name for inflammation of the blood vessels

they are characterised on weather they affect small, medium or large vessels

Question 2

name the types of vasculitis that affect the small vessels

Answer 2

• Henoch-Schoenlein purpura
• Eosinophilic Granulomatosis with Polyangiitis (EGPA) (Churg-Strauss syndrome)
• Microscopic polyangiitis (MPA)
• Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Question 3

name the types of vasculitis that affect the medium sized vessels

Answer 3

• Polyarteritis nodosa
• Eosinophilic Granulomatosis with Polyangiitis (EGPA) (Churg-Strauss syndrome)
• Kawasaki Disease

Question 4

name the types of vasculitis that affect the large vessels

Answer 4

• Giant cell arteritis (more likely in over 50s)

* Takayasu’s arteritis (more likely in under 50s)

Question 5

What are the generic features/presentation that apply to most types of vasculitis

Answer 5

• Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.
• Joint and muscle pain
• Peripheral neuropathy
• Renal impairment
• Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)
• Anterior uveitis and scleritis
• Hypertension

Question 6

what markers are usually raised in vasculitis

Answer 6

inflammatory markers CRP and ESR

Question 7

what are the two types of Anti-neutrophil cytoplasmic antibodies (ANCA)

Answer 7

p- ANCA aka anti-MPO antibodies

c- ANCA aka anti-PR3 antibodies

Question 8

which type of vasculitis is associated with p-ANCA (MPO antibodies)

Answer 8

Microscopic polyangiitis

Churg-Strauss syndrome (aka Eosinophilic Granulomatosis with Polyangiitis (EGPA) )

Question 9

which type of vasculitis is associated with c-ANCA (PR3 antibodies)

Answer 9

Wegener’s granulomatosis

Question 10

give some examples of the different steroids that are used in the management of vasculitis

Answer 10

• Oral (i.e. prednisolone)
• Intravenous (i.e. hydrocortisone)
• Nasal sprays for nasal symptoms
• Inhaled for lung involves (e.g. Churg-Strauss syndrome)

Question 11

give some examples of immunosuppressants that can be used to manage vasculitis

Answer 11

• Cyclophosphamide
• Methotrexate
• Azathioprine
• Rituximab and other monoclonal antibodies

Question 12

which two of the vasculitis mainly affect children

Answer 12

henoch-schoenlein purpura (HSP) and Kawasaki

Question 13

which antibody is involved in HS purpura

Answer 13

IgA vasculitis and so inflammation occurs due to immunoglobulin A deposits in the blood vessels of the affected organs such as the skin, kidneys and GI tract

Question 14

what is henoch-schoenlein purpura (HSP) often triggered by

Answer 14

an upper airway infection eg tonsillitis or gastroenteritis

most common in children under 10

Question 15

what are the four classic features of henoch-schoenlein purpura (HSP)

Answer 15

purpura (100%), joint pain (75%), abdominal pain (50%) and renal involvement (50%). HSP affects the kidneys in about 50% of patients, causing an IgA nephritis.

management is typically supportive

Question 16

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) most associated with lung and skin but can affect other organs such as kidney

how does it often present

Answer 16

with severe asthma in late teenage years or adulthood

A characteristic finding is elevated eosinophil levels on the full blood count.

Question 17

Microscopic polyangiitis is a small vessel vasculitis. what is the main feature of microscopic polyangiitis

Answer 17

renal failure

can also affect the lungs causing SOB and haemoptysis

Question 18

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

it is a small vessel disease that affects the respiratory tract and kidneys. How does this classically present in exams

Answer 18

saddle shaped nose due to a perforated nasal septum which causes a dip halfway down the nose

other causes; 
epistaxis 
crusty nasal secretions
hearing loss 
sinusitis 

in the lungs;
cough, wheeze and haemoptysis

Question 19

what can Granulomatosis with polyangiitis (Wegener’s granulomatosis) often be misdiagnosed as on a CXR

Answer 19

pneumonia

Question 20

Polyarteritis nodosa (PAN) is a medium vessel vasculitis. what is it most associated with

Answer 20

hepatitis B but can also occur without a clear cause or with hepatitis C and HIV.

Question 21

polyarterittis nodosa is associate with a rash known as

Answer 21

livedo reticularis. This is a mottled, purplish, lace like rash

Question 22

Kawasaki disease is a medium vessel vasculitis that affects young children, typically under 5. There is no clear cause

what are the clinical features

Answer 22

• Persistent high fever > 5 days
• Erythematous rash
• Bilateral conjunctivitis
• Erythema and desquamation (skin peeling) of palms and soles
• “Strawberry tongue” (red tongue with prominent papillae)

Question 23

what is the key complication with Kawasaki disease and the treatment

Answer 23

coronary artery aneurysm

aspirin and IV immunoglobulins
known as muco-cutaneous lymphadenopathy

Question 24

Takayasu’s arteritis is a form of large vessel vasculitis

where does it mainly affect

Answer 24

the aorta and its branches
it also affects the pulmonary arteries

can swell and form aneurysms or become narrowed and blocked which leads to its other name of ‘pulseless disease’

Question 25

how does Takayasu’s arteritis usually present

Answer 25

before the age of 40 years with non-specific systemic symptoms, such as fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope.

It is diagnosed using CT or MRI angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease