Vasculitis

Question 1

What are the different categories of vasculitis?

Answer 1

• immune complex small vessel vasculitis
• ANCA-associated small vessel vasculitis
• medium vessel vasculitis
• large vessel vasculitis

Question 2

What types of vasculitis is immune complex small vessel vasculitis?

Answer 2

• cryoglobulinemic vasculitis
• IgA vasculitis
• hypocomplementemic urticarial vasculitis
• anti-GBM disease

Question 3

What type of vasculitis is in ANCA-associated small vessel vasculitis?

Answer 3

• microscopic polyangiitis
• granulomatosis with polyangiitis
• eosinophilic granulomatosis with polyangiitis

Question 4

What vasculitis is in medium vessel vasculitis/

Answer 4

• polyarteritis nodosa
• Kawasaki disease

Question 5

What vasculitis is in large vessel vasculitis?

Answer 5

• takayasu arteritis
• giant cell arteritis

Question 6

What sign might you see on examination in giant cell arteritis?

Answer 6

thickened, non-pulsatile temporal artery

Question 7

Define giant cell arteritis

Answer 7

systemic vasculitis that affects the aorta and its major branches

Question 8

Describe a typical clinical presentation of giant cell arteritis

Answer 8

• headache (temporal with pain on palpation, subacute onset, constant with little relief from analgesics)
• visual symptoms
• jaw claudication (pain when eating/talking)
• polymyalgia reumatica symptoms (shoulder/pelvic girdle pain)
• constitutional symptoms

Question 9

What are the complications of giant cell arteritis?

Answer 9

• visual loss (irreversible loss, acute ischaemic neuropathy, sudden painless loss - can be preceded by amaurosis fugal)
• large vessel vasculitis (stenosis and aneurysm - stroke risk)
• CVA (obstruction/occlusion of internal carotid a./vertebral a.

Question 10

What investigations would you want to do in giant cell arteritis?

Answer 10

• temporal artery biopsy (gold standard)
• temporal artery US
• MRI
• PET CT (good to check if large vessel involvement - aneurysm)

Question 11

How would you medically treat giant cell arteritis?

Answer 11

• prednisolone 1mg/kg/day (discontinue by 12-18months)
• • aspirin 75mg (if risk for stroke)
• methotrexate/ mycophenolate mofetil/ tocilizumab (steroid-sparing therapy) for relapses

Question 12

What are the causes of cutaneous vasculitis?

Answer 12

• idiopathic
• drugs
• infection (HCV, HBV, gonococcus, meningococcus, HIV)
• secondary RA/CTD/PBC/UC
• malignancy (esp lymphoma)
• manifestation of small/medium ANCA vasculitis

Question 13

What important tests should you do if you suspect vasculitis?

Answer 13

• CRP/ESR
• urinalysis (kidney function)
• FBC, LFTs, Us and Es

Question 14

Describe the signs of Henoch Schonlein purpura

Answer 14

• purpuric rash on buttocks and thighs
• urticarial rash, petechiae, ecchymoses, ulcers
• arthralgia/arthritis (lower limb)

Question 15

What are the complications of Henoch Schonlein purpura?

Answer 15

• GI: pain, bleeding, diarrhoea, intussusception (rare)
• renal: IgA nephropathy
• urinary: orchitis
• CNS (rare)

Question 16

What is the management and prognosis for Henoch Schonlein purpura?

Answer 16

• often no treatment
• corticosteroids for certain complications (testicular torsion, GI, arthritis)
• self-limiting with small minority relapsing within 12 months

Question 17

Describe granulomatosis with polyangiitis (GPA)

Answer 17

granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract and often a pauci-immune glomerulonephritis (no immune deposit in glomeruli)

Question 18

What is the classical triad of symptoms and signs of GPA?

Answer 18

• upper airway symptoms: rhinitis, chronic sinusitis, chronic otitis media, saddle nose deformity, nasal septal perforation
• renal symptoms: pauci-immune glomerulonephritis
• lower respiratory symptoms: parenchymal nodules and cavitation, alveolar haemorrhage (haemoptysis)

Question 19

What factors would increase your clinical suspicion of vasculitis?

Answer 19

• classical presentation
• constitutional symptoms
• multi-system disease
• repeat GP/hospital visits
• disease ‘not behaving’ as it should

Question 20

Describe the immunology of ANCA vasculitis

Answer 20

• autoantibodies against cytoplasmic constituents of neutrophils and monocytes
• cANCA with PR3 (neutrophils) very suggestive of GPA
• pANCA with strong MPO suggestive of MPA (or EGPA)
• positive not always indicative of ANCA

Question 21

How useful is ANCA detection?

Answer 21

• not helpful as diagnostic tool (not all positive ANCE = ANCA vasculitis)
• useful prognostic information (increased ANCA = increased chance of relapse)
• can monitor for for early signs of relapse)

Question 22

What is the treatment for vasculitis?

Answer 22

1. remission induction (prednisolone + cyclophosphamide/rituximab or methotrexate/mycophenolate - milder drugs)
   - switch off vasculitis activity
   - higher dose = higher toxicity
   - 3-6m
2. remission maintenance (azathioprine/ methotrexate/ rituximab)
   - prevent relapse
   - lower drug toxicities
   - more prolonged therapy

Question 23

What are the risks of cyclophosphamide treatment?

Answer 23

• cytopenias
• malignancy
• infertility
• rituximab is safer and no risk of infertility