Vasculitis

Overview

Vasculitis is an umbrella term for a series of conditions represented by inflammation of the blood vessels. Its effects can be transient or cause damage to the vasculature. 

It can happen in isolation (primary), or secondary due to infection or in association with another condition such as rheumatoid arthritis.

Classification

• Large-vessel vasculitides:
  • Temporal (giant cell) arteritis
  • Takayasu’s arteritis
• Medium-vessel vasculitides:
  • Polyarteritis nodosa
  • Kawasaki disease
• Small-vessel vasculitides:
  • ANCA-associated vasculitides
    • Granulomatosis with polyangiitis (Wegener’s granulomatosis)
    • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
    • Microscopic polyangiitis
  • Immune complex vasculitides
    • Henoch-Schönlein purpura
    • Anti-glomerular basement membrane disease (Goodpasture’s syndrome)
  • Behçet’s disease

Aetiology

• Idiopathic – around 50%
• Infection (e.g. Henoch-Schönlein purpura or septic vasculitis)
• Inflammatory disease (e.g. systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Crohn’s disease, and ulcerative colitis
• Drug-induced (e.g. sulfonamides, penicillins, quinolones, NSAIDs etc.)
• Neoplastic –  (e.g. lymphoproliferative disorders or paraproteinaemia)

Presentation

Some general features can help point to the type of vasculitis the patient is experiencing:

• Small-vessel vasculitides:
  • Palpable purpura
  • Tiny papules
  • Splinter haemorrhages
  • Urticaria
  • Vesicles
  • Rarely livedo reticularis
  • Renal involvement
• Medium-sized vessel vasculitides:
  • Ulcers
  • Digital infarcts
  • Nodules
  • Livedo reticularis
  • Hypertension if there is damage to the renal vessels
  • Papular and necrotic skin lesions
• Large vessel vasculitides:
  • End-organ damage e.g. TIA/stroke
  • Hypertension
  • Aneurysms
  • Dissection with or without haemorrhage or rupture

Investigations

Overview

Initial tests may include:

• C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR):
  • Elevated
• Anti-neutrophil cytoplasmic autoantibodies (ANCA):
  • Associated with certain types of vasculitis, such as granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.
• Urea and electrolytes (U&Es):
  • Vasculitis may involve the kidneys leading to renal dysfunction
• Urinalysis:
  • Vasculitis may involve the kidneys leading to renal dysfunction
• Biopsy of the affected tissue:
  • Can help with confirming the likely diagnosis

Management

Overview

The mainstay of management in vasculitides involves the use of immunosuppression with glucocorticoids (such as prednisolone) with the addition of other immunosuppressants such as cyclophosphamide.