First Things First (assess & treat for the following)

  • Vasculitic syndromes are characterized by inflammation & necrosis of blood vessel walls, resulting in narrowing or occlusion of the lumen or formation of aneurysms.
  • Sequelae of anatomic changes include ischemia, infarction, turbulent flow and/or bleeding from aneurysmal rupture.
  • Blood vessels anywhere throughout the body can be affected, but the extent & site of vessel involvement vary considerably in different cases & syndromes.
  • Vascular involvement tends to be irregular & segmental rather than continuous.
  • The vasculitides have in common vascular inflammation, yet there are different types & presentations. They could be isolated (skin, retina, CNS, etc.); they can be systemic (multiple organ involvement); they can be acute w/ all lesions at same stage or chronic w/ lesions at different stages of inflammation.
  • Prognosis is variable: some have a benign self-limited course (ie, Henoch-Schönlein purpura [HSP]); others can be life- or organ-threatening w/ fatal prognosis if untreated (ie, Wegener’s).
    • Pathogenic mechanisms are varied & complex. Vessel damage can be mediated by:
      • Immune complex deposition
      • Antiendothelial cell antibodies
      • Antibodies against lysosomal enzymes
      • Pathogenic cellular immune response & granuloma formation
      • Infection agents, tumor cells, drugs
    • Classification: vasculitides are grouped according to size of predominant blood vessels involved.
      • Large vessel vasculitis
        • Takayasu’s arteritis (TA)
        • Giant cell (temporal) arteritis (GCA)
      • Medium vessel vasculitis
        • Polyarteritis nodosa (PAN)
        • Kawasaki’s disease
        • Isolated CNS vasculitis
      • Small vessel vasculitis; Also known as hypersensitivity/allergic vasculitis or leukocytoclastic vasculitis.
        • Anti-GBM disease
        • ANCA-associated syndromes: Wegener’s granulomatosis (WG), microscopic polyangiitis, Churg-Strauss syndrome (CSS), pauci-immune necrotizing crescentic glomerulonephritis (GN)
        • Cryoglobulinemic vasculitis (hepatitis C)
        • HSP
        • Primary cutaneous leukocytoclastic angiitis: drugs, infectious, malignancy, autoimmune
        • Hypocomplementemic urticarial vasculitis
        • Thromboangiitis obliterans (Buerger’s disease) not a true vasculitis

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Last updated: May 17, 2010