Vasculitis is a topic covered in the 5-Minute Emergency Consult.

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  • Injury to the walls of blood vessels from inflammation:
    • Ischemia and necrosis
    • Aneurysms and hemorrhage
    • Immunopathologic mechanisms:
      • Deposition of circulating antigen–antibody complex and complement fixation
      • Cell-mediated hypersensitivity
      • Granulomatous tissue reaction from persistent inflammation and formation of epithelioid giant cells
  • The vasculitides represent a wide group of disorders:
    • Multisystem disease with constitutional symptoms and inflammatory lab indices
    • Secondary to another disorder or trigger, or primary if vasculitis is the principal feature and the cause is unknown
    • Multiple factors determine presentation:
      • The size of the affected blood vessels
      • The specific distribution, severity, and duration of the inflammation
      • Degree of permeability or occlusion of the affected vessels
  • 1 out of 2,000 adults has some form of vasculitis


  • Classification is evolving and is increasingly based on presence or absence of antineutrophil cytoplasmic antibodies (ANCA).
  • Traditional classification is based on vessel size.
  • Large vessel vasculitides:
    • Temporal (giant cell) arteritis:
      • Granulomatous arteritis of the aorta and its major branches often involving the temporal artery
      • Patients >50 yr
    • Takayasu arteritis:
      • Granulomatous inflammation of the aorta and its major branches
      • Usually occurs in patients <50 yr
  • Medium vessel vasculitides:
    • Polyarteritis nodosa (PAN):
      • Small- and medium-sized arteritis
      • Common distribution includes vessels supplying the muscles, joints, intestines, nerves, kidneys, and skin
      • Most common in middle age
    • Kawasaki disease (mucocutaneous lymph node syndrome):
      • Coronary arteries are often involved and involves large-, medium-, and small-sized arteries
      • Usually occurs in children
    • Isolated CNS vasculitis
  • Small vessel vasculitides:
    • Granulomatosis with polyangiitis (Wegener granulomatosis):
      • Necrotizing vasculitis affecting small- to medium-sized vessels
      • Granulomatous inflammation of the upper and lower respiratory tract and glomerulonephritis
    • Microscopic polyangiitis:
      • Necrotizing affecting small vessels
      • Glomerulonephritis is very common.
      • Pulmonary capillaritis often occurs.
    • Churg–Strauss syndrome (allergic granulomatosis):
      • Small- and medium-sized arteries
      • Mainly lungs, GI, and nerves
      • Can also involve heart, skin, and kidney
    • Henoch–Schönlein purpura:
      • Most patients <20 yr
    • Buerger disease (thromboangiitis obliterans):
    • Hypersensitivity vasculitis:
      • Recurring inflammation and thrombosis of small and medium arteries and veins of the hands and feet
      • Typically between 20 and 40 yr and male
    • Secondary vasculitides:
      • Bacterial infections:
        • Streptococcal, tuberculous, staphylococcal, Lyme disease, leprosy
      • Viral infections:
        • Hepatitis B or C, CMV, HSV, HIV
      • Rickettsial infections
      • Drug related
      • Connective tissue disease:
        • Systemic lupus erythematosus
        • Rheumatoid arthritis
        • Behçet disease
      • Malignancy:
        • Hairy cell leukemia
        • Lymphoma
      • Mixed cryoglobulinemia
      • Goodpasture syndrome
      • Serum sickness

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