Vasculitis and anti-GBM associated diseases

Vasculitis is an inflammation of vessel walls. The inflammation may be acute or chronic, and can lead to necrosis, fibrosis or thrombosis.

In vasculitis, any type of vessel in any tissue may be affected. The clinical manifestations of vasculitis are therefore protean and can affect many different organs.

Nomenclature of systemic vasculitides

(adopted by the Chapel Hill Consensus Conference)

Large vessel vasculitis:

  • Giant cell (temporal) arteritis
  • Takayasu's arteritis

Medium-sized vessel vasculitis:

  • Polyarteritis nodosa
  • Kawasaki's disease

Small vessel vasculitis:

  • Glomerulonephritis with polyangiitis (GPA), formerly called Wegener's granulomatosis*
  • Eosinophilic glomerulonephritis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome*
  • Microscopic polyangiitis*
  • Henoch-Schönlein purpura
  • Essential cryoglobulinaemic vasculitis
  • Cutaneous leukocytoclastic angiitis

*ANCA-associated vasculitides