Vasculitis - general

Question 1

How is vasculitis defined?

Answer 1

It is an inflammatory disorder if blood vessel walls, causing destruction (aneurysm/rupture) or stenosis.

Question 2

Where can it affect?

Answer 2

It can affect the vessels of any organ; presentation therefore depends on which organ is involved.

Question 3

How is vasculitis classified?

Answer 3

• Large vessel: Giant Cell Arteritis, Takayasu’s arteritis
• Medium vessel: Polyarteritis nodosa, Kawasaki disease
• Small vessel:
  1) ANCA +ve vasculitis which has a predilection for the respiratory tract and kidneys; it includes p-ANCA associated microscopic polyangiitis, glomerulonephritis and Churg-Strauss syndrome and c-ANCA associated Wegener’s Granulomatosis
  2) ANCA -ve vasculitis includes Henoch-Schönlein purpura, Goodpasture’s syndrome and cryoglobinaemia

Question 4

What are the symptoms of vasculitis?

Answer 4

Different vasculitides preferentially affect different organs,, causing different patterns of symptoms.
Often may only present with overwhelming fatigue with raised ESR/CRP.

Consider vasculitis in any unidentified multi system disorder.

If presentation does not fit clinically or serologically into specific category consider malignancy-associated vasculitis.

A sever vasculitis flare is a MEDICAL EMERGENCY - organ damage may occur rapidly, e.g. critical renal failure

Question 5

What tests can you perform to help diagnose a vasculitis?

Answer 5

• ESR/CRP will be raised
• ANCA may be positive
• Increased creatine in renal failure
• Urine: check proteinuria, haematuria, casts
• Angiography +/- biopsy for diagnosis

Question 6

What is the management of vasculitis?

Answer 6

Large vessel: steroid in most cases
Medium/small: steroids and cyclophosphamide (15mg/kg)
Aziothioprine may be useful as steroid sparing maintenance treatment.

Question 7

What are the general features of vasculitis?

Answer 7

• SYSTEMIC - fever, malaise, Wertzolls, arthralgia, myalgia
• SKIN - purpura, ulcers, livedo reticularis, nailbed infarcts, digital gangrene
• EYES - episcleritis, scleritis, visual loss
• ENT - epistaxis, nasal crusting, stridor, deafness
• PULMONARY - haemoptysis and dyspnoea (due to pulmonary haemorrhage)
• CARDIAC - Angina due to MI (coronary arteritis), heart failure, pericarditis
• GI - Pain or perforation (infarcted viscous), malabsorption form chronic ischaemia
• RENAL - Hypertension, haematuria, proteinuria, casts and renal failure (renal cortical infarcts; glomerulonephritis in ANCA +ve vasculitis)
• NEUROLOGICAL - stroke, fits, chorea, psychosis, confusion, impaired cognition, altered mood. Arteritis of the vasa nervorum (arterial supply to the peripheral nerves) may cause mononeuritis multiplex or a sensorimotor polyneuropathy
• GU - Orchitis