How is vasculitis defined?
It is an inflammatory disorder if blood vessel walls, causing destruction (aneurysm/rupture) or stenosis.
Where can it affect?
It can affect the vessels of any organ; presentation therefore depends on which organ is involved.
How is vasculitis classified?
- 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
What are the symptoms of vasculitis?
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
What tests can you perform to help diagnose a vasculitis?
- ESR/CRP will be raised
- ANCA may be positive
- Increased creatine in renal failure
- Urine: check proteinuria, haematuria, casts
- Angiography +/- biopsy for diagnosis
What is the management of vasculitis?
Large vessel: steroid in most cases
Medium/small: steroids and cyclophosphamide (15mg/kg)
Aziothioprine may be useful as steroid sparing maintenance treatment.
What are the general features of vasculitis?
- 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
